先天性肥厚性幽门狭窄卡哈尔间质细胞和突触的免疫组织化学研究

目的：研究先天性肥厚性幽门狭窄（CHPS）幽门卡哈尔间质细胞发育和突触形成情况。方法：使用SP免疫组织化学技术对32例CHPS和10例正常对照者进行检测。结果：对照组，c-kit抗体阳性细胞大量分布于肌间神经丛神经节细胞周围，中等量分布于环肌层，少量分布于纵肌层，它们相互联结形成网状；肌间神经丛内可见大量突触素抗体表达，纵肌和环肌层可见中等量突触素抗体表达。CHPS组，c-kit抗体阳性细胞在肌间神经丛和环肌层明显减少，它们形成的网络被破坏；肌层突触素抗体表达显著减少，而肌间神经丛无明显变化。结论：CHPS有卡哈尔间质细胞发育异常和突触形成异常，这些可能是CHPS出现症状的原因。     

一氧化氮在先天性肥厚性幽门狭窄肥厚肌和血中改变的研究

目的：测定一氧化氮（ＮＯ）在先天性肥厚性幽门狭窄（ＣＨＰＳ）肥厚肌中的改变，血中含量及术前术后的改变，探讨ＣＨＰＳ的发生和病理生理改变，方法：用还原性辅酶Ⅱ（ＮＡＤＰＨｄ）组织化学染色测定组织中一氧化氮合酶（ＮＯＳ）含量，用比色法测定血中ＮＯ含量，２１例ＣＨＰＳ术中取幽门肥厚之肌层；５例非幽门疾病幽门肌层。结果：ＣＨＰＳ组肥厚之环状肌层无ＮＡＤＰＨｄ（＋）神经纤维染色，偶见扭曲，变形，染色线的神经     

新生儿幽门环肌切开术的改进

自1997～1999年，我院对23例新生儿先天性肥厚性幽门狭窄行双轨式幽门环肌切开术，术后均取得满意效果。手术方法和结果：在幽门部血管较少处平行相距0．5cm纵形切开两个切口（图1）。十二指肠端以白线为界，胃端至移行部。长约2．0～3．5cm，深度0、4~0．6cm。两个纵切口不要一次完全切断肥厚的幽门环肌，在切开幽门环肌约2/3深度以后，形成的长条肌瓣会自然向上膨隆。再在两纵行切口形成的长条肌瓣上，从中间横断，切开浆膜及部分环肌。这样就形成了两个方形的小皮瓣，此时粘膜并求膨出。用拇指在十二指肠壁上轻轻牵动，使皮瓣移动，然后…     

经腹腔镜幽门环肌切开术

目的总结腹腔镜下行幽门环肌切开手术的操作要点和经验。方法回顾性分析2003年12月以来应用腹腔镜治疗先天性肥厚性幽门狭窄27例病例资料及手术情况。结果27例全部手术成功,无1例中转开腹及二次手术。结论腹腔镜下行幽门环肌切开术治疗先天性肥厚性幽门狭窄是安全可靠的。     

先天性肥厚性幽门狭窄的合并畸形

先天性肥厚性幽门狭窄是常见的消化道畸形 ,少数有伴发畸形。我院 1990～ 2 0 0 0年手术的 76例新生儿先天性肥厚性幽门狭窄中 ,3例合并幽门前瓣膜 ,1例合并肠旋转不良 ,现报告如下。资料和方法3例合并幽门前瓣膜者中 ,1例是先天性肥厚性幽门狭窄 ,行幽门环肌切开术后仍持续呕吐 ,胃肠减压无胆汁 ,钡餐检查提示胃输出道梗阻 ,疑幽门环肌切开不完全 ,于术后第 9ｄ二次手术 ,术中检查幽门环肌切开彻底 ,但胃窦部触诊似有膈膜 ,胃内气体不能迅速排空 ,遂切开胃窦部 ,证实幽门前有膈膜型不完全闭锁 ,行膈膜切除幽门成形术 ,术后呕吐缓解 ,患儿痊…     

腹腔镜与开腹幽门环肌切开术的前瞻性比较研究

目的研究比较腹腔镜幽门环肌切开术（LP）和开腹幽门环肌切开术（OP）治疗先天性幽门肥厚性狭窄的疗效及免疫功能的变化。方法自2003年4月-2006年7月将72例先天性幽门肥厚性狭窄患儿随机分成二组（LP组及OP组各36例），比较二组麻醉时间、手术时间、术后进食时间及术后并发症，监测二组术前、术后第一天、术后第三天的外周血T淋巴细胞亚群、C反应蛋白（CRP）及白细胞介素-6（IL-6）和肿瘤坏死因子（TNF）的变化并行对比研究。结果二组麻醉时间、手术时间、术后进食时间差异无统计学意义，OP组术后并发症要略多于LP组，比较二组术前、术后第一天、术后第三天的外周血T淋巴细胞亚群、CRP及IL-6和TNF的变化差异无统计学意义。结论腹腔镜幽门环肌切开术（LP）和开腹幽门环肌切开术（OP）治疗先天性幽门肥厚性狭窄的临床效果相近，二组患儿免疫功能的变化无显著性差异。腹腔镜幽门环肌切开术是一种稳定、可靠的手术，对于治疗先天性肥厚性幽门狭窄的效果满意。     

腹腔镜下幽门环肌切开术治疗先天性肥厚性幽门狭窄62例

目的总结腹腔镜下幽门环肌切开术治疗小儿先天性肥厚性幽门狭窄的临床经验,探讨其手术技巧和相关并发症的防治。方法回顾性分析2010年3月至2014年7月本院经腹腔镜行幽门环肌切开治疗的62例先天性肥厚性幽门狭窄患儿临床资料。结果62例中,59例顺利完成手术,术中3例幽门黏膜损伤中转开腹手术,术后1例并发脐部戳孔疝,经修补治愈,1例术后仍有呕吐,行开放手术证实为肌层切开厚度不够所致。所有病例均无呕吐及肠粘连、肠梗阻等并发症发生。结论腹腔镜下行幽门环肌切开术治疗小儿先天性肥厚性幽门狭窄疗效确切,术中损伤小,术后瘢痕小,但需要较高的腹腔镜操作技术。     

梅克尔憩室肠壁神经和平滑肌细胞的分布研究

目的 了解梅克尔憩室(MD)肠壁神经和平滑肌病理组织学改变,探讨MD及其并发症的发病机制.方法 收集2004年1月至2006年7月手术切除的44例MD标本,另取6例死于与肠道和神经系统无关疾病的足月新生儿尸检小肠标本为对照,采用常规HE染色计数肠壁肌间神经丛和丛内神经节细胞数,S-100蛋白、神经元特异性烯醇化酶(NSE)和平滑肌肌动蛋白(α-SMA)采用免疫组织化学染色(SABC法).结果 MD的肠壁肌间神经丛密度与对照组相比无统计学意义(P＞0.05),但丛内神经节细胞数、单个肌间神经丛的平均面积及其平均神经丛长轴长度均较对照组减少,而平均光密度值比对照组增加.肌间神经丛的平均面积与其平均吸光度呈负相关(r=一0.215,P＜0.05),且上述病理改变以症状组和伴有异位胃黏膜的MD尤为突出.MD的肠壁环肌层和纵肌层α-SMA表达减低,环肌层肥厚;而距憩室5 cm处肠壁肌间神经丛的各项指标的改变均减轻,但是环肌层仍明显肥厚,α-SMA染色亦减轻.结论 肠壁神经丛和平滑肌细胞的变化可能参与了MD及其并发症的发生.     

腹腔镜幽门环肌切开术治疗先天性肥厚性幽门狭窄

目的 介绍腹腔镜治疗先天性肥厚性幽门狭窄的初步经验。方法 １９９８年以来应用腹腔镜治疗先天性肥厚性幽门狭窄１５例。平均年龄４０天,平均体重３．８ｋｇ．在气管插管＋单次硬膜外麻醉下,脐上皮肤小切口,Ｖｅｒｅｓｓ针穿刺入腹,注入ＣＯ２,建立气腹,压力为１０－１４ｍｍＨｇ（１．３３－１．８６ｋＰａ）,此外置－４ｍｍ套管,放放腹腔镜,直视下左右上腹各置一套管,放入操作器械,完成幽门环肌切开。     

脐褶切口行幽门环肌切开术

将30例先天性肥厚性幽门狭窄患儿分成二组,一组经右上腹膜直肌切口,另一组经济褶切口行幽门环肌切开术。比较手术时间、术后发生呕吐例数及持续时间、切口感染的发生率和术后住院日,经统计学处理两组无显著性差异,表明脐褶切口行幽门肌环切开术是安全可行的。     

先天性肥厚性幽门狭窄致病因素及发病机制

先天性肥厚性幽门狭窄是婴幼儿常见的消化道畸形.国内报道先天性肥厚性幽门狭窄的发病率为1/1000～1/10000,男性患者多于女性,男女比例约为5∶1.先天性肥厚性幽门狭窄时幽门括约肌肥厚、增生,导致胃出口机械性梗阻,引起频繁的呕吐.严重的呕吐影响患儿生长发育,甚至危及生命.关于先天性肥厚性幽门狭窄的具体发病机制尚未完全明确,可能与幽门括约肌发育、神经支配、神经细胞发育和胃肠激素水平异常有关.近年来,相关研究表明环境因素和遗传因素对先天性肥厚性幽门狭窄的发病均有一定作用.该文就先天性肥厚性幽门狭窄致病因素及发病机制的研究进展作一综述.     

Pathogenesis of infantile hypertrophic pyloric stenosis: recent progress

Although infantile hypertrophic pyloric stenosis (IHPS) is the most common condition requiring surgery in the first few months of life, its pathogenesis is not fully understood. Reviews of the recent progress in the pathogenesis of IHPS show: (1) there is increasing evidence to suggest that smooth-muscle cells in IHPS are not properly innervated; (2) because non-adrenergic, non-cholinergic nerves are mediators of smooth-muscle relaxation, it is likely that the absence of these nerves in pyloric muscle is the cause of excessively contracted hypertrophic circular pyloric muscle; (3) there are abnormal amounts of extracellular matrix proteins in hypertrophic pyloric muscle. Circular muscle cells in IHPS are actively synthesizing collagen, and this may be responsible for the characteristic “firm” nature of the pyloric tumor; and (4) the increased expression of insulin-like growth factor-I, transforming growth factor-ß1, and platelet-derived growth factor-BB and their receptors in hypertrophic pyloric muscle suggests that increased local synthesis of growth factors may play an important role in smooth-muscle hypertrophy in IHPS.     

先天性肥厚性幽门狭窄手术方式研究进展

先天性肥厚性幽门狭窄是新生儿和小婴儿常见的消化道先天畸形，幽门肌切开术是其最有效的治疗方法，包括几种术式：经典的开腹下幽门肌切开术对患儿的机体损伤较严重，有麻醉意外、术后感染、皮肤疤痕等风险；经腹腔镜下幽门肌切开术易引起幽门肌切开不全、侵入性操作损伤等弊端，经胃镜下幽门肌切开术对新生儿损伤小、没有手术疤痕、操作简单、手术并发症少、术后喂养恢复快，经口内镜行黏膜下幽门肌切开术为一种新术式，需要进一步研究。     

小儿先天性肥厚性幽门狭窄合并肠旋转不良的临床特点

目的 探讨小儿先天性肥厚性幽门狭窄合并肠旋转不良的临床特点.方法 回顾性分析2003年1月至2009年12月于我科治疗的6例先天性肥厚性幽门狭窄合并肠旋转不良患儿的临床资料.结果 通过胃肠透视及超声检查,4例患儿术中确诊该病并经1次手术解决,2例术前仅诊断为先天性肥厚性幽门狭窄,行幽门肌切开术,再次出现呕吐后行2次手术发现肠旋转不良.所有病例预后良好.结论 先天性肥厚性幽门狭窄合并肠旋转不良较为少见,病因不清,先天性肥厚性幽门]狭窄的症状易掩盖肠旋转不良的临床表现而贻误其诊断.胃肠透视对于诊断该病有重要意义,彩超检查有良好的应用价值,及时的诊断治疗可获得良好的治疗效果.     

New insights into the pathogenesis of infantile pyloric stenosis

Infantile hypertrophic pyloric stenosis (IHPS) is the most common surgical cause of vomiting in infants. Despite numerous hypotheses, the aetiopathogenesis of IHPS is not fully understood. Genetic, extrinsic and hormonal factors have been implicated in the pathogenesis of the disease. Furthermore, abnormalities of various components of the pyloric muscle such as smooth muscle cells, growth factors, extracellular matrix elements, nerve and ganglion cells, synapses, nerve supporting cells, neurotransmitters and interstitial cells of Cajal have been reported. Recently, genetic studies have identified susceptibility loci for IHPS and molecular studies have concluded that smooth muscle cells are not properly innervated in IHPS.     

Defective cholinergic innervation in pyloric muscle of patients with hypertrophic pyloric stenosis

The etiology of hypertrophic pyloric stenosis (HPS) is not known. Many investigators have studied the myenteric plexus and muscle in HPS, but there are no reports of cholinergic nerve distribution in this condition. A histochemical technique for the detection of acetylcholinesterase (AChE) has been used to study cholinergic nerve distribution. Recently, it has been shown that the development and survival of cholinergic neurons in the peripheral and central nervous system depend on the presence of nerve growth factor (NGF) and its receptor (NGFR). We examined pyloric muscle from 18 patients with HPS and 10 controls using monoclonal antibody to NGFR and AChE histochemistry. Myenteric plexus displayed strong NGFR and AChE reactivity. Quantitative assessment of immunoreactivity in nerve fibres within pyloric muscle demonstrated selective absence of NGFR and AChE-positive nerve fibres in the circular and longitudinal muscle of HPS, whereas these fibres were in abundance in the muscle of controls. These findings suggest that NGFR is an important neurotrophic factor in the maintenance of cholinergic neuronal function and that NGFR deficiency, resulting in defective cholinergic innervation of pyloric muscle, may have an important role in the pathogenesis of HPS.     

两孔法腹腔镜下幽门环肌切开术270例

目的探讨两孔法腹腔镜治疗先天性肥厚性幽门狭窄（CHPS）的临床价值。方法回顾性分析本院采用两孔法腹腔镜治疗的270例CHPS患儿的临床资料，对其并发症及再手术原因进行总结。结果270例中，2例改用三孔法完成手术，3例中转开腹手术，其余265例均经两孔法完成手术。全部病例术后呕吐情况明显减少，两孔法手术的患儿住院时间缩短。结论两孔法腹腔镜下幽门环肌切开术是治疗先天性肥厚性幽门狭窄的一种行之有效的微创手术方法，可临床推广应用。     

Increased insulin-like growth factor-I mRNA expression in pyloric muscle in infantile hypzertrophic pyloric stenosis

The etiology of infantile hypertrophic pyloric stenosis (IHPS) is unknown. Insulin-like growth factor-I (IGF-I) is a polypeptide hormone that elicits various biological activities (cellular growth, replication, and differentiation) by binding to its receptors. IGF-I has been suggested to play an important role in both gastrointestinal (GI) maturation and smooth-muscle-cell (SMC) hypertrophy. Full-thickness muscle biopsy specimens were obtained from 8 IHPS patients (age range 14–64 days, mean 28.1 days) at pyloromyotomy and from 8 age-matched controls (15–60 days, mean 33.8 days) without GI disease at autopsy. In-situ hybridization was performed using an IGF-I-specific and digoxigenin (DIG)-labeled oligonucleotide probe and visualized by nitroblue tetrazolium staining. In normal controls, IGF-I mRNA expression was absent or weak in both circular and longitudinal smooth-muscle layers of pyloric muscle. In contrast, the pyloric muscle in IHPS patients demonstrated strong IGF-I mRNA expression in the circular smooth-muscle layer and moderate expression in the longitudinal smooth-muscle layer. The increase in IGF-I mRNA in pyloric muscle in IHPS suggests that SMCs are actively synthesizing IGF-I, contributing to the development of pyloric muscle hypertrophy.     

Increased local synthesis of epidermal growth factors in infantile hypertrophic pyloric stenosis

Infantile hypertrophic pyloric stenosis (IHPS) is characterized by hypertrophy of the pyloric muscle. The growth of smooth muscle cells is regulated by several growth factors. Epidermal growth factor (EGF) and heparin-binding EGF-like growth factor are potent mitogens for smooth muscle cells. In the present study, we investigated immunohistochemical localization of EGF and EGF-related peptides and EGF mRNA expression in pyloric smooth muscle cells to determine whether the EGF family is involved in the process of pyloric muscle hypertrophy in IHPS. Pyloric muscle biopsy specimens were obtained at the time of pyloromyotomy from 10 patients with IHPS. Control material included 10 pyloric muscle specimens taken at autopsy from age-matched cases without evidence of gastrointestinal disease. Indirect immunohistochemistry was performed using the avidin-biotin-peroxidase complex method with anti-EGF, anti-EGF receptor, and anti-heparin-binding EGF-like growth factor antibody. In situ hybridization was performed using digoxigenin-labeled EGF-specific oligonucleotide probe. The pattern of immunoreactivity in pyloric muscle with EGF, EGF receptor, and heparin-binding EGF-like growth factor was similar in all specimens. There was a marked increase in EGF, EGF receptor, and heparin-binding EGF-like growth factor immunoreactivity and EGF mRNA expression in smooth muscle cells in pyloric circular and longitudinal muscle from patients with IHPS compared with control specimens. These data suggest that the upregulated local synthesis of EGF and EGF-related peptides in pyloric muscle may play a critical role in the development of pyloric muscle hypertrophy in IHPS.     

Congenital hypertrophic pyloric stenosis

Congenital hypertrophic pyloric stenosis, an important cause of intractable vomiting in infants is diagnosed clinically and confirmed ultrasonographically. Other useful interventions are plain radiography and berium study. Differential diagnosis includes pylorospasm and gastroesophageal reflux. Management protocol includes correction of dehydration and electrolyte imbalance and either Fredet Ramstedt pyloromyotomy or medical treatment with atropine sulphate. Atropine is initially given intravenously till vomiting is controlled and then orally at double the effective I.V. done for another 3 weeks. Atropine sulphate is generally well tolerated and side effects are few like tachycardia, raised SGPT and hyperthermia. Atropine sulphate is very effective, cheap, safe and perhaps more acceptable treatment option for CHPS.