非开腹式经肛门结肠拖出术治疗小儿先天性巨结肠症

目的 报道既不用开腹,也不需腹腔镜,在微创的情况下实现对小儿先天性巨结肠症的根治术式。方法 采用经肛门斜行切断直肠（前高后低）,处理直肠、结肠系膜,拖出病变肠管并予以切除,结、直肠I期斜面吻合的方法,治疗短段型（5例）及普通型（18例）巨结肠共23例。结果 全部患者手术获得成功,术后无须扩肛,随方1～12个月,排例正常,未出现不良并发症。结论 非开腹式,经肛门拖出并切除病变结肠,是一种全新的手术路径。它不实现对巨结肠根治的同时,获得了最佳的美容效果,并降低了一定的术后并发症。     

关注先天性巨结肠术后便秘复发

自从丹麦医生 Hirschsprung 报告首例先天性巨结肠症至今100多年以来,先天性巨结肠的外科治疗获得显著进步。1948年 Swenson 等［1］采用开腹直肠切除、结肠拖出与肛管吻合术,开创了先天性巨结肠经肛门拖出（Pull-through）的经典外科术式,先后又有诸如 Duhamel、Soave、Reheibin 等多种术式得到临床应用,并获得较好的治疗效果［2］。上世纪90年代,先天性巨结肠外科治疗进入新的历史时期,Georgeson 等［3］于1995年报告腹腔镜辅助经肛门拖出术;特别是 Torre 等［4］于1998年首次报道单纯经肛门结肠拖出术,使先天性巨结肠手术进一步简化,创伤更小,恢复更快,并在世界范围内获得广泛认可和应用。中国医科大学附属盛京医院对早期行 Swenson 改良术的患儿远期（8～16年）排便功能评估结果显示,Sewenson 术后远期排便功能优良率达84．4％［5］。而基于 Swenson 原创经肛拖出理念改进的单纯经肛拖出和（或）腹腔镜辅助下经肛拖出术,由于采用更为简捷的手术路径,显著减轻绝大多数先天性巨结肠患儿的手术创伤和瘢痕形成,从而获得较传统手术后更高的生活质量［6］。虽然先天性巨结肠的现代外科治疗取得不断进步,但先天性巨结肠手术后便秘复发仍屡有报告。在一项对先天性巨结肠术后长达8～24年的随访研究中,发现14．3％的患者术后再次出现便秘［7］。有人统计 Duhamel 术后远期随访结果,便秘复发率为15％,Soave 术为16％,而巨结肠同源病术后便秘复发率更高［8］。国内有文献报告先天性巨结肠经 Swenson 根治术后,便秘症状复发可高达47．9％［9］。因此,先天性巨结肠术后便秘复发问题需引起临床重视。     

先天性巨结肠术后直肠肛管向量测压的研究

目的：应用直肠肛管向量测压技术评估先天性巨结肠患儿术后肛门括约肌功能。方法：利用直肛管向量测压技术，对42例先天性巨结肠患儿术后及21例正常儿进行肛门括约肌功能的评估。结果：根据临床症状将患儿分为污便组、便秘组和排便功能良好组。巨结肠患儿术后肛管静息压力及向量容积均显著低于正常儿（P＜0．01），污便组的静息压力及向量容积明显低于排便功能良好组，对称指数无明显变化，14．3％恢复了直肠肛门抑制反射。结论：先天性巨结肠患儿术后肛管最大压力及向量容积下降。直肠肛管向量测压技术是评估先天性巨结肠患儿术后肛门括约肌功能较客观全面的方法。     

经脐腹腔镜结肠拖出术治疗先天性巨结肠症

目的 介绍经脐腹腔镜下拖出术治疗先天性巨结肠的手术方法以及临床手术经验.方法 回顾2009年6月至9月,对9例先天性巨结肠患儿采用经脐腹腔镜下拖出术进行治疗.患儿平均年龄为31.9个月(年龄范围在1～99个月),平均体重为16.2 kg(体重范围在4.7～25 kg).患儿脐窝处分别置入3个5 mm trocar,在腹腔镜镜头监控下,使用特制弯曲手柄型腹腔镜操作杆分离相应肠系膜及血管.扩肛,分离直肠肌肉与黏膜,然后将病变肠管呈袖套式拖出肛门外切除,行结肠肛门心形吻合术.记录术前各项检查以及手术相关数据.对手术患儿进行随访,记录术后并发症的发生情况.结果 平均手术时间为155 min(时间范围在110～185 min),平均失血量为40 ml(失血范围在10～100 ml).没有患儿术中转为传统腹腔镜手术或者开腹手术.无一例患儿术中出现腹部血管、肠管、输尿管、输精管损伤.患儿术后3 d进流食,术后7 d可出院.在随访期间,详细记录患儿术后并发症和恢复情况:1例患儿术后16 d出现小肠结肠炎,经过抗感染等对症治疗后好转;其余患儿均未出现术后并发症(吻合口狭窄、吻合口漏、便秘、污粪、大便失禁、腹泻、小肠结肠炎).术毕脐窝稍红肿,术后30d患儿复诊未见明显手术瘢痕.结论 经脐腹腔镜拖出术来治疗先天性巨结肠具有简单易行、美观的优点,适用于长段型巨结肠患儿.     

先天性巨结肠微创化手术治疗研究

目的探讨先天性巨结肠微创化手术治疗的方法。方法回顾性分析2004年1月至2007年1月本院收治的68例先天性巨结肠患儿的病例资料。年龄2个月至3岁，其中短段型12例，普通型40例，长段型10例，全结肠型6例。手术方式包括直肠肌条切除术、单纯经肛门结肠拖出术、腹腔镜辅助下巨结肠根治术。结果7例行直肠肌条切除术，35例行单纯经肛门结肠拖出术，20例行腹腔镜辅助下巨结肠根治术，6例行开腹手术。均治愈出院，出院后随访4个月至4年，63例排便正常，5例仍存在便秘。结论进一步规范儿童先天性巨结肠的微创治疗，制定儿童先天性巨结肠微创化治疗标准是提高儿童先天性巨结肠疗效的保证。     

经肛门先天性巨结肠根治术16例

目的 介绍一种经肛门行先天性巨结肠根治的手术方式并评价其疗效.方法 16例确诊为先天性巨结肠的患儿均采用经肛门前高后低大斜面吻合的方法完成根治手术.结果 术后排便正常14例,吻合口狭窄1例,吻合口出血1例.结论 经肛门前高后低大斜面吻合巨结肠根治术具有创伤小,手术时间短,术后恢复快,腹部无手术疤痕等优点,但应严格掌握手术适应症.     

The choice of operative procedures for Hirschsprung's disease allied diseases in children

目的 对先天性巨结肠同源病的手术处理方式做探讨.方法 2008年1月至2010年12月45例巨结肠同源病行根治术,平均年龄(35.5±5)个月,其中节细胞减少症(HG)33例(有既往手术史4例,12.1％),肠神经元发育不良症(IND) 12例(有既往手术史4例,33.33％).所有患儿均在术后1、3、6及12个月进行随访,记录患儿术后排便功能并与术前结果相比较.结果 HG组33例中17例(52％)经开腹手术,16例(48％)腹腔镜辅助或经肛门拖出术.结肠切除范围:左半切除28例(84.8％),次全切除5例(15.2％);12例IND组全部经开腹手术,结肠左半切除4例(33.3％),次全切除8例(66.7％).术后随访:所有患儿便秘症状消失,无手术死亡及严重并发症发生.不同术式组中均有少数患儿持续存在污粪现象,1年期随访经肛门手术显著高于开腹手术(P＜0.05).出现术后污粪患儿施行肛管直肠测压术前肛管静息压(66.5±11.67) mmHg,术后3个月为(52.17±0.31)mmHg较术前明显下降,但至术后6个月～1年后测压为(58±5.7)mmHg,与术前相比均无显著差异.结论 同源病的手术应根据不同病理类型和临床情况选择手术方式:原发性HG病变可采用直接经肛门拖出或腹腔镜辅助游离术.既往曾经肛门直肠手术或年长、晚期患儿应采取保留肛管直肠括约肌形态和功能完整性的术式.IND患儿均需行根治性次全切除术,不主张采用简单的经肛门拖出术式而应采用盆腔内的低位吻合术式.经肛门或腹腔镜辅助经肛门拖出术式组1年期随访时污粪率要高于非拖出术组,左半切或次全切方式对术后是否污粪无影响.     

经肛门改良Soave术和经腹手术治疗先天性巨结肠疗效比较

目的探讨经肛门改良Soave术和经腹手术治疗先天性巨结肠的临床疗效。方法2001～2007年作者收治年龄〉3岁的先天性巨结肠患儿41例，其中28例采取经肛门改良Soave术，24例经腹手术，分析两组患儿术后并发症以及排便功能。结果两组并发症的发生率比较，经肛门改良Soave术明显少于经腹手术，先天性巨结肠相关性肠炎（Himchsprung’s associatedentero colitis，HAEC）的发生率低于经腹手术，差异有统计学意义。两组排便控制能力以及大便形态方面比较，经肛门改良Soave术优于经腹手术。结论经肛门改良Soave术治疗先天性巨结肠安全可行，疗效优于经腹手术，但术后仍有诸多并发症，特别在排便控制方面，需要大宗病例长期随访的研究来评估。     

先天性巨结肠经肛门Ⅰ期根治术后便秘的再手术探讨

目的分析经肛门SoaveⅠ期拖出根治术治疗先天性巨结觞后使秘的病例，探讨其原因、再手术的指征和方法。方法2000年来经肛门SoaveⅠ期拖出根治术治疗先天性巨结肠术后发生便秘的患儿17例；分为2组。保守治疗组共4例，占23．5%；手术组13例，占76．5％；手术组又分为开腹和再次经肛门直接拖出手术治疗2组；其中开腹手术组4例，经肛门直接拖出治疗9例。对手术时间、合并症、术后便失禁、便秘的情况进行分析随访。结果保守治疗组有3例经洗肠、排便训练及年龄增加便秘情况由1次／2～4d，转为1次／1～2d，无合并症；1例治疗2年因反复肠炎，顽固性便秘转手术治疗。经肛门再次直接拖出治疗10例和腹部小切口辅助经肛门拖出治疗4例，无手术并发症。患儿术后1个月随访时：排便2～6次／d，半年时：排便1～3次／d；术后2年随访排使±1—2次／d，无肛门狭窄。仅1例偶有污裤。结论经肛门SoaveⅠ期拖出时．因视野小、拖出困难；容易遗留先神经节细胞的结肠，是术后便秘的主要原因。经肛门SoaveⅠ期拖出根治术具有腹腔干扰少和粘连少的优点，因此再次手术时，仍可采用此方法。有腹腔肠粘连的患儿可腹部切口辅助下经肛门Soave Ⅰ期直接拖出治疗。     

腹腔镜辅助技术在先天性巨结肠手术中的应用价值

目的探讨腹腔镜辅助技术在不同类型先天性巨结肠手术中发挥的作用。方法159例先天性巨结肠患儿接受手术治疗。短段型25例，常见型114例，长段型20例。其中单纯经肛门Soave拖出手术32例；腹腔镜Soave-Georgeson手术70例；腹腔镜直肠肛管背侧纵切心形吻合术57例。主要观察手术过程、手术时间、住院费用、并发症、术后排便功能。结果在短段型和常见型患儿，经肛门Soave手术时间与腹腔镜Soave-Georgeson手术和腹腔镜直肠肛管背侧纵切心形吻合术时间相同，但肛门解剖时间前者比后两者明显长。腹腔镜组住院费用比经肛门Soave手术组高，术后并发症、排便优良率、直肠肛管反射恢复率、肛管静息差、肛管高压区长度均无明显差异。长段型患儿腹腔镜Soave-Georgeson手术和直肠肛管背侧纵切心形吻合术均明显比经肛门Soave手术时间长、住院费用高，但腹腔镜操作不可缺少。结论对于短段型和常见型巨结肠根治术，常规应用腹腔镜没有必要，但腹腔镜辅助经肛门拖出手术是更全面的技术，能显著缩短肛门解剖时间，游离更长的病变结肠，更容易判断无神经节细胞肠段范围，观察结肠肛门吻合后肠管是否扭转、腹腔出血等。     

Allergic factors associated with the development of asthma and the influence of cetirizine in a double-blind, randomised, placebo-controlled trial: First results of ETA®

There is a common progression known as the allergic march from atopic dermatitis to allergic asthma. Cetirizine has several antiallergic properties that suggest a potential effect on the development of airway inflammation and asthma in infants with atopic dermatitis. Methods. Over a two year period, 817 infants aged one to two years who suffered from atopic dermatitis and with a history of atopic disease in a parent or sibling were included in the ETAC^® (Early Treatment of the Atopic Child) trial, a multi-country, double-blind, randomised, placebo-controlled trial. The infants were treated for 18 months with either cetirizine (0.25mg/ kg b.i.d.) or placebo. The number of infants who developed asthma was compared between the two groups. Clinical and biological assessments including analysis of total and specific IgE antibodies were performed. Results. In the placebo group, the relative risk (RR) for developing asthma was elevated in patients with a raised level of total IgE (≥ 30 kU/I) or specific IgE (≥ 0.35 kUA/I) for grass pollen, house dust mite or cat dander (RR between 1.4 and 1.7). Compared to placebo, cetirizine significantly reduced the incidence of asthma for patients sensitised to grass pollen (RR = 0.5) or to house dust mite (RR = 0.6). However, in the population that included all infants with normal and elevated total or specific IgE (intention-to-treat - ITT), there was no difference between the numbers of infants developing asthma while receiving cetirizine or placebo. The adverse events profile was similar in the two treatment groups. Discussion. Raised total IgE level and raised specific IgE levels to grass pollen, house dust mite or cat dander were predictive of subsequent asthma. Cetirizine halved the number of patients developing asthma in the subgroups sensitised to grass pollen or house dust mite (i.e. 20% of the study population). In view of the proven safety of the drug, we propose this treatment as a primary pharmacological intervention strategy to prevent the development of asthma in specifically sensitised infants with atopic dermatitis.     

�¶�֢��ϵ�ϰ���ע��ȱ�ݶද�ϰ������ڵ�ת��

孤独症谱系障碍(autistic-spectrum disorders,ASDs)近年来患病率逐年攀升至1%左右,其症状往往伴随终生,成为严重威胁儿童健康和发展的神经发育性疾患;注意缺陷多动障碍(attention deficit hyperactivity disorder,ADHD)是儿童期最常见的精神障碍,国内报道患病率为4.13%～5.83%,其症状可延续至青少年期,甚至到成年期[1]。这两类精神障碍在成年期的临床表现、共患病、治疗策略和预后与儿童期有哪些不同呢?本文通过回顾相     

EXCITING NEW TREATMENT APPROACHES FOR PATHYPHYSIOLOGIC MECHANISMS OF SICKLE CELL DISEASE

During the past several decades, our understanding of the complex pathophysiology of vasoocclusion associated with sickle cell disease has improved greatly. Interaction of genes, hemoglobin molecules, red cell membrane and metabolic changes, cell-cell interactions and cell-plasma interactions, red cell adhesion to vascular endothelium, activation of coagulation, and vascular reactivity play a role in vaso occlusion. Penicillin prophylaxis of pneumococcal infections and appropriate use of blood transfusions and other supportive measures improved survival of sickle cell patients. Hydroxyurea made a major impact on sickle cell therapy when it was shown to decrease acute painful episodes, acute chest syndrome, and the need for blood transfusion in adults. Significant experience in the use of hydroxyurea has been accumulated in older children. The benefits and risks of hydroxyurea for younger children and long-term risks in all patients will be evaluated in future investigations. Other promising therapies include butyrate compounds, clotrimazole, magnesium supplementation, poloxamer 188, antiadhesion agents, anticoagulant approaches, and nitric oxide. Hemopoietic transplantation remains the only curative therapy. However, several transgenic mouse models are available for studies of gene therapy or other treatment approaches on biochemical, cellular, and pathologic effects of mutant genes.     

Infiltrations of Epstein-Barr virus-carrying cells in granular lymphocyte-proliferative disorders corresponding to chronic active Epstein-Barr virus infection

A 21-year-old man with granular lymphocyte-proliferative disorders (GLPD) associated with chronic active Epstein-Barr virus (EBV) infection is described. Chromosomal analyses revealed several clonal abnormalities and two of them were mainly repetitious. High copy numbers of monoclonal EBV genome were also detected in the proliferative large granular lymphocytes (LGLs), indicating the monoclonal expansion of EBV-infected LGLs. The patient had an indolent course for several years, and there was no evidence of infiltrations of his bone marrow until the end stage. At autopsy, microscopic studies revealed marked infiltrations of LGL in the liver and spleen, and the infiltrating cells were NK-cell immunophenotype. The infiltrated LGLs showed latency I.     

LUTEINIZING HORMONE RECEPTOR MUTATIONS IN DISORDERS OF SEXUAL DEVELOPMENT AND CANCER

Human male sexual development is regulated by chorionic gonadotropin (CG) and luteinizing hormone (LH). Aberrant sexual development caused by both activating and inactivating mutations of the human luteinizing hormone receptor (LHR) have been described. All known activating mutations of the LHR are missense mutations caused by single base substitution. The most common activating mutation is the replacement of Asp-578 by Gly due to the substitution of A by G at nucleotide position 1733. All activating mutations are present in exon 11 which encodes the transmembrane domain of the receptor. Constitutive activity of the LHR causes LH releasing hormone-independent precocious puberty in boys and the autosomal dominant disorder familial male-limited precocious puberty (FMPP). Both germline and somatic activating mutations of the LHR have been found in patients with testicular tumors. Activating mutations have no effect on females. The molecular genetics of the inactivating mutations of the LHR are more variable and include single base substitution, partial gene deletion, and insertion. These mutations are not localized and are present in both the extracellular and transmembrane domain of the receptor. Inactivation of the LHR gives rise to the autosomal recessive disorder Leydig cell hypoplasia (LCH) and male hypogonadism or male pseudohermaphroditism. Severity of the clinical phenotype in LCH patients correlates with the amount of residual activity of the mutated receptor. Females are less affected by inactivating mutation of the LHR. Symptoms caused by homozygous inactivating mutation of the LHR include polycystic ovaries and primary amenorrhea.     

Resurgence of measles in Singapore: profile of hospital cases

OBJECTIVE: To ascertain the profile of cases of measles seen at a general hospital during a recent outbreak that occurred despite a measles vaccination program. METHODOLOGY: A retrospective study from January 1991 to March 1998. All patients with measles (ICD code 055. 9) seen at the emergency unit or as inpatients were included. RESULTS: There were 87 cases identified. The diagnosis was clinical in all and proven serologically in 71%. Eighty-five per cent of the cases occurred between January 1997 and March 1998. There was a bi-modal age distribution with peaks in the very young (相似文献   

Personality profiles and heart rate variability (vagal tone) in children with recurrent abdominal pain

The aim of the study was to explore psychological factors and autonomic activity in children with recurrent abdominal pain and to compare them with those in a control group of healthy children. The Personality Inventory for Children was used for assessment of developmental, emotional and psychosocial factors in 25 children with recurrent abdominal pain (age, 7-15 y). Parasympathetic and sympathetic functions in these children and in 23 healthy control subjects (age, 7-13 y) were also investigated, non-invasively using a computerized polygraph. Vagal tone (parasympathetic function) was indexed by calculation of respiratory sinus arrhythmia in beats/min. Skin conductance (sympathetic function) was recorded by the constant current method. On the Personality Inventory for Children, 16 patients had high scores on somatic concern. Several patients had scores in the clinical range for depression, withdrawal and anxiety, but the mean scores for these personality profile scales were well within the normal range of healthy children. Interestingly, there was a spike on the L (Lie)-scale for most of the patients and 15 patients had scores above or close to the clinical cut-off value. As compared with the scores in healthy children, vagal tone and sympathetic tone were normal. Conclusion: Many children with recurrent abdominal pain have scores in the clinical range for depression, withdrawal, anxiety and L-scale indicating coping problems, denial and a trend towards somatic concern that may contribute to the evolution of abdominal pain. Autonomic nerve activity was not disturbed in these children.     

Overcoming surfactant inhibition with polymers

Inhibition of the function of pulmonary surfactant in the alveolar space is an important element of the pathophysiology of many lung diseases, including meconium aspiration syndrome, pneumonia and acute respiratory distress syndrome. The known mechanisms by which surfactant dysfunction occurs are (a) competitive inhibition of phospholipid entry into the surface monolayer (e.g. by plasma proteins), and (b) infiltration and destabilization of the surface film by extraneous lipids (e.g. meconium-derived free fatty acids). Recent data suggest that addition of non-ionic polymers such as dextran and polyethylene glycol to surfactant mixtures may significantly improve resistance to inhibition. Polymers have been found to neutralize the effects of several different inhibitors, and can produce near-complete restoration of surfactant function. The anti-inhibitory properties of polymers, and their possible role as an adjunct to surfactant therapy, deserve further exploration.     

Understanding infant formula

The World Health organisation recommends breast feeding infants for the first six months of life. When this breast feeding does not occur either through parental choice or medical need, infant formulas will be required. There is a bewildering array of formulas on the UK market for many different requirements. When faced with an unsettled infant many parents (and healthcare professionals) will experiment with the infant formula available and then attend the paediatric clinic looking for help and advice. It is therefore essential that paediatricians understand what milks are available and what the key differences between different products are. This review attempts to provide a simple guide through many of the formulations currently available in the UK; and offers advice for the dietary management of the child with extra calorie requirements, infants with cow's milk protein allergy, gastro oesophageal reflux disease, apparent unresolved hunger and infantile colic. Whatever the underlying condition, there is likely to be an infant formula that is suitable in this generation of ever expanding formulations.