14例小儿乙状结肠冗长症手术治疗分析

目的总结小儿乙状结肠冗长症的诊断、治疗、疗效及短期随访经验。方法2005年至2012年我们收治17例乙状结肠冗长症患儿,其中经手术治疗14例,保守治疗3例。回顾性分析14例手术治疗患儿临床资料。结果14例手术治疗患儿中,13例行经肛门结肠拖出术,1例行经腹Soave术。术后随访8例,最短半年,最长4年,均无吻合口瘘、吻合口狭窄及便秘等并发症。3例行药物、灌肠、饮食疗法等保守治疗6个月,症状好转,2~3 d排便一次。结论小儿乙状结肠冗长症的诊断主要依据临床表现及钡灌肠检查,确诊后应积极临床治疗。首选保守治疗,但症状改善缓慢,保守治疗无效或效果不满意者宜转手术治疗,手术首选经肛门结肠拖出术,损伤小,恢复快,无瘢痕,疗效明显。     

经肛门结肠拖出根治小儿乙状结肠冗长症

目的总结经肛门结肠拖出根治小儿乙状结肠冗长症的手术过程、疗效及短期随访。方法我院2000～2005年经该术式治疗小儿乙状结肠冗长症19例，乙状结肠冗长症有典型的便秘症状，钡灌肠显示19例乙状结肠比正常对照组平均长25cm；直肠肛管测压显示直肠肛管松弛反射存在，呈“W”型特殊波型；组织化学检查无乙酰胆碱酯酶阳性的副交感神经纤维。结果19例经肛门拖出切除结肠肠管20～52cm，平均切除结肠肠管28cm。均于术后2～3d开始进食，术后1个月随访每日排便6～8次，其中15例行钡灌肠检查未见结肠扩张，24h无钡剂潴留。6个月随访（17例）每日排便1～3次，无污粪。结论经肛门结肠拖出根治小儿乙状结肠冗长症手术安全有效，具有创伤小、操作简单、近期疗效良好的特点。     

小儿乙状结肠冗长症的诊断和治疗

目的 乙状结肠冗长症是引起小儿便秘的主要原因之一，为进一步提高该病的诊治水平，对该病的诊断和治疗作进一步探讨。方法 总结我院近７年来（１９９１－１９９７年）收治的１２例乙状结肠冗长症的临床资料。乙状结肠冗长症有典型的便秘症状。钡灌肠显示１２例乙状结肠比正常对照组平均长２２ｃｍ。     

改良Soave术与Sarasola-Klose内痔环切术联合治疗儿童肛门、直结肠弥漫性血管畸形

目的 介绍改良Soave术结合Sarasola-Klose内痔环切术联合治疗儿童肛门、直肠、结肠血管畸形.方法 回顾分析2000年至2008年收治的12例小儿肛门、直肠、乙状结肠血管畸形患儿的临床资料,就其诊断和手术治疗进行分析.结果 根据病史、纤维结肠镜检查、钡剂灌肠、腹部CT和MRI检查,临床诊断为弥漫性肛门、直肠、乙状结肠血管畸形.11例患儿接受改良经腹Soave术,肛周黏膜层及黏膜下血管切除采用Sarasola-Klose内痔环切术式(改良Whitehead术).1例患儿仅接受常规经腹Soave术.12例患儿术后随访3个月～8年,1例仍有少量间歇性便血,余均无便血、便秘,1例污粪.结论 血管畸形累及肛门、直肠甚至乙状结肠的病例少见,肠镜、CT、MRI是诊断的主要方法.改良Soave术结合Sarasola-Klose内痔环切术是安全、彻底、有效的治疗方法.     

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。     

小儿乙状结肠冗长症21例诊治分析

目的总结小儿乙状结肠冗长症的临床特点、诊断及治疗方法。方法回顾性分析我院近7年来收治的21例乙状结肠冗长症患儿的病例资料,并结合文献讨论临床表现及诊疗方法。结果21例均获痊愈,经3个月～6年随访,患儿排便功能恢复,发育状况良好。结论小儿乙状结肠冗长症以慢性便秘和间歇性腹痛为主要症状;诊断主要根据钡剂灌肠检查,部分病例应与先天性巨结肠、特发性巨结肠鉴别;治疗方法首选非手术疗法,无效时行手术治疗。     

经肛门改良Swenson和Soave术治疗先天性巨结肠比较

目的比较经肛门改良Swenson术和Soave术治疗先天性巨结肠的疗效及优缺点。方法回顾性分析经肛门手术治疗的50例先天性巨结肠患儿临床资料，其中短段型11例，普通型35例，长段型4例；行经肛门改良Swenson术21例，经肛门改良Soave术29例。比较两组患儿手术相关因素、术后并发症及排便功能情况。结果两种术式术前灌肠时间、术中出血量、术后住院天数及出院时排便情况比较，无统计学意义（P〉0．05），而手术时间比较，前者较后者短（P〈0．05）。两组术后并发症的发生率分别为19．0％和37．9％，术后6个月时排便优良率为95．2％和89．6％。结论经肛门改良Swenson术和改良Soave术创伤小，恢复快，疗效好，但经肛门Swenson术较经肛门Soave术操作更简单方便，并发症明显减少；两者手术适应证均可适当放宽。     

肠无神经节细胞症患儿术后肛门直肠测压的应用研究

目的：探讨经肛门 Soave 巨结肠根治术后患儿排便功能及直肠肛管测压的应用价值。方法回顾性分析经术后病理检查证实的61例肠无神经节细胞症（Hirschsprung＇s disease,HD）患儿临床资料,均行经肛门 Soave 巨结肠根治术,其中腹腔镜辅助手术16例。术后平均随访1年（3个月至2年）,根据手术年龄分为婴儿组（0～1岁）及幼儿组（1岁以上）。对患儿术后大便控制能力、并发症情况及肛门直肠测压结果进行比较。结果婴儿组术后排便控制情况与幼儿组比较,差异无统计学意义（P ＞0.05）。肛门直肠测压检查显示术前直肠肛管抑制反射总阴性率为95.1％（58／61）,术后均未恢复;两组直肠静息压、肛管静息压比较,差异无统计学意义（P ＞0.05）。结论经肛门 Soave 巨结肠根治术治疗小儿肠无神经节细胞症,术后可获得良好的排便控制功能,可作为首选术式;肛门直肠测压的术前诊断价值较高,术后短期随访作用有限。     

经肛门婴幼儿先天性巨结肠症根治术

外科治疗小儿先天性巨结肠症,传统手术方法需开腹手术或经腹会阴手术,以及经改进的腹腔镜辅助经会阴手术都对患儿创伤较大,且并发症较多。我院于2001年6月～2004年11月采用不开腹Ⅰ期经肛门巨结肠根治术治疗婴幼儿先天性巨结肠症13例,效果良好,现报告如下。1资料与方法1.1病例资料本组13例中,男9例,女4例,年龄4个月～3岁。术前根据病史、临床表现、肛门指诊及钡灌肠X线片等检查明确诊断。术中及术后病理检查证实先天性巨结肠症诊断。其病理类型:短段型4例,常见型9例,均无严重并发症。术前准备给予温盐水清洁回流灌肠10～15d,术前3d给予肠道…     

微创手术与经肛门Soave术治疗先天性巨结肠的个性化选择

目的 根据病情选择单纯改良经肛门Soave术、辅助小切口手术或辅助腹腔镜手术对先天性巨结肠进行个性化治疗,并评价其安全性和有效性.方法 2002年1月至2010年12月,对137例先天性巨结肠患儿采用改良一期经肛门Soave术（Ⅰ组）,并根据年龄、影像学检查、病变类型选择个性化手术方案,其中单纯经肛门手术81例（ⅠA组）,辅助左下腹皮横纹小切口手术13例（ⅠB组）,腹腔镜辅助手术43例（ⅠC组）.并与1999年1月至2001年12月间112例（Ⅱ组）实施传统经肛门Soave术的患儿进行比较,其中单纯经肛门手术87例（ⅡA组）,开腹辅助手术35例（ⅡB组）.统计学分析采用两样本t检验、卡方检验以及Fisher检验,P＜0.05为差异有统计学意义.结果 ①Ⅰ组和Ⅱ组患儿术前临床资料相似,差异无统计学意义.②与ⅡA组相比,ⅠA组术后恢复快,并发症少,肛门功能恢复好.③与ⅡB组相比,ⅠB与ⅠC组术后并发症少,肛门功能恢复好,而ⅠB与ⅠC组无差异.结论 根据不同病情个性化选择单纯改良经肛门Soave术、辅助小切口手术或辅助腹腔镜手术,可有效减少手术并发症,提高生活质量.     

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.     

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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     

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.     

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.     

Bibliometric data: a disaster for many non-American biomedical journals

Bibliometric data published by the Institute of Scientific Information in Philadelphia (ISI), and which was previously discussed in Acta Paediatrica , has increasingly been used despite all the relevant and severe criticism that has been raised against this method of evaluating individual research results and grading scientific journals. It is obvious that the present trend regarding the use of bibliometric data as a basis for priorities and funding of research and for the promotion of individual scientists favours American-oriented research projects at the expense of those that are based on concepts of predominantly European relevance.

Conclusion: For the future of non-American research, it is important that no single super-power, i.e. the USA, should dominate scientific priorities. The condition for efficient European competition is that European Centres with high levels of competence for creative research and training of scientists from all over the world are established. In addition, it is important that the results of European research are published in prestigious European journals, as was the situation before World War II.     

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.     

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.     

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.