腹腔镜与开腹手术治疗先天性肥厚性幽门狭窄安全性及预后的Meta分析

目的:比较腹腔镜幽门肌切开术（laparoscopic pyloromyotomy,LP）与开腹幽门肌切开术（open pyloromyotomy,OP）治疗先天性肥厚性幽门狭窄（congenital hypertrophic pyloric stenosis,CHPS）的安全性及预后,为临床治疗方案的制定提供循证依据...     

腹腔镜幽门环肌切开术学习曲线的研究

目的评估不同阶段腹腔镜幽门环肌切开术治疗先天性肥厚性幽门狭窄的手术效果，探讨该术式的学习曲线。方法2001～2006年应用腹腔镜技术治疗先天性肥厚性幽门狭窄120例，以每30例手术为一个学习曲线阶段，比较4个阶段手术时间、术中术后并发症发生率、中转开腹率及术后住院时间，分析各阶段手术效果。结果4个阶段患儿年龄和性别组成比较，无统计学意义（P〉0．05）。早期30例平均手术时间35±12min，2例因术中损伤幽门黏膜予中转开腹修复术，2例合并脐部戳孔疝；后期90例平均手术时间（15±6）min（P〈0．01），无术中及术后并发症发生，无中转开腹。各组术后住院时间比较。无统计学意义（P〉0．05）。结论腹腔镜幽门肌切开术的学习曲线约30例，随着手术经验的逐步积累，手术时间明显缩短，手术并发症亦明显减少。     

经腹腔镜先天性肥厚性幽门狭窄手术

目的 探讨小儿先天性肥厚性幽门狭窄腹腔镜微创手术操作要点及经验教训。方法 回顾性分析本组20例患儿全麻下经腹腔镜行幽门环肌切开术。结果 18例一次手术成功，1例术中中转开腹，1例3d后二次手术。全部病例随访2月，预后较好。结论 腹腔镜手术治疗先天性肥厚性幽门狭窄是腔镜技术在新生儿外科领域的成功运用，掌握操作要点，可推广使用。     

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。     

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。     

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。     

微孔腹腔镜治疗先天性肥厚性幽门狭窄的体会

目的探讨微孔腹腔镜治疗先天性肥厚性幽门狭窄的优越性。方法采用1个5mm电视腹腔镜微孔、2个3mm操作微孔行幽门环肌切开术16例。结果微孔腹腔镜手术无1例中转开腹，平均手术时间25min。术后12h拔除胃管，开始少量喂奶，无手术并发症发生。结论腹腔镜幽门环肌切开手术与开腹手术相比有一定的优越性，手术打击小，切口小，术后恢复快，手术瘢痕小，外观美观，患儿家长易接受。     

腹腔镜与开腹手术治疗先天性肥厚性幽门狭窄的比较

目的 比较腹腔镜与常规开腹手术治疗先天性肥厚性幽门狭窄的疗效.方法 2008年3月至2011年10月,我们分别应用腹腔镜及常规开腹手术治疗先天性肥厚性幽门狭窄163例,从手术时间、术后呕吐、并发症等方面比较两种手术的优缺点.结果 87例行常规开腹手术,76例行腹腔镜手术.两种手术方法在术后呕吐次数、手术时间、术后并发症上比较,差异无统计学意义.腹腔镜组有2例穿孔,术中中转开腹行修补术.术后呕吐与体重及年龄比较,差异有统计学意义（P＜0.01）.结论 腹腔镜及常规开腹手术均是治疗先天性肥厚性幽门狭窄的安全可靠的治疗方法,腹腔镜手术具有损伤小、伤口美观等优点.     

腹壁牵引法与CO2气腹法腹腔镜幽门环肌切开术的比较

目的 比较腹壁牵引法与CO2气腹法两种幽门环肌切开术的优缺点,探讨腹壁牵引法幽门环肌切开术的应用价值.方法 回顾分析2006年3月至2007年9月,我院普外科收治先天性肥厚性幽门狭窄病例66例,其中21例行腹壁牵引法腹腔镜幽门环肌切开术,45例行CO2气腹法腹腔镜幽门环肌切开术,比较两组患儿术中血气分析及生命体征监测的各项指标、术后并发症、住院时间及治疗费用.结果 CO2气腹法组气腹10 min后,pH值、PCO2、HCO3-浓度较气腹前变化明显,差异有统计学意义(P<0.05),MBP、HR较术前有升高,但差异尤统计学意义(P0.05),SPO2改变不明显.腹壁牵引法组在Veress针穿刺10 min后各个指标较术前均无明显变化.两组比较手术用时及术后偶发呕吐比例差异无统计学意义(P0.05),术后进食时间、术后呼吸道感染发生比例、住院天数、治疗费用差异均有统计学意义(P<0.05).结论 腹壁牵引法腹腔镜幽门环肌切开术较传统气腹法腹腔镜手术更简便、经济,并发症相对少,有一定的应用价值.     

腹壁牵引法与CO2气腹法腹腔镜幽门环肌切开术的比较

目的 比较腹壁牵引法与CO2气腹法两种幽门环肌切开术的优缺点,探讨腹壁牵引法幽门环肌切开术的应用价值.方法 回顾分析2006年3月至2007年9月,我院普外科收治先天性肥厚性幽门狭窄病例66例,其中21例行腹壁牵引法腹腔镜幽门环肌切开术,45例行CO2气腹法腹腔镜幽门环肌切开术,比较两组患儿术中血气分析及生命体征监测的各项指标、术后并发症、住院时间及治疗费用.结果 CO2气腹法组气腹10 min后,pH值、PCO2、HCO3-浓度较气腹前变化明显,差异有统计学意义(P<0.05),MBP、HR较术前有升高,但差异尤统计学意义(P0.05),SPO2改变不明显.腹壁牵引法组在Veress针穿刺10 min后各个指标较术前均无明显变化.两组比较手术用时及术后偶发呕吐比例差异无统计学意义(P0.05),术后进食时间、术后呼吸道感染发生比例、住院天数、治疗费用差异均有统计学意义(P<0.05).结论 腹壁牵引法腹腔镜幽门环肌切开术较传统气腹法腹腔镜手术更简便、经济,并发症相对少,有一定的应用价值.     

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.     

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.     

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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.     

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.     

alpha -SMOOTH MUSCLE ACTIN DISTRIBUTION IN THE PULMONARY VASCULATURE COMPARING HYPOPLASTIC AND NORMAL FETAL LUNGS

We investigated the intra-acinar pulmonary vascular muscularization in the developing human fetal lung between the 17th and 24th gestational weeks, that is, during the canalicular phase of lung development. Fifteen hypoplastic and 25 normal developed lungs were included in this study using monoclonal alpha -smooth muscle (sm) actin antibodies for smooth muscle detection. Computer-aided image analysis was performed for morphometrical measurements and statistical evaluation. Alphasm-actin-immunoreactive intra-acinar vessels down to a luminal diameter of less than 10 mu m were detected in hypoplastic as well as in normally developed lungs. Crucial differences presented as follows: significantly higher density of intra-acinar vessels, especially due to alpha -sm-actin-negative vessels less than 30 mu m in luminal diameter, in the control group; significantly higher alpha -sm-actin immunoreactivity per section unit as well as per vessel in the hypoplastic lung group. As suggested by others, alpha-sm-actin-positive cells of the intra-acinar vessel wall in the developing human lung were demonstrated to be smooth muscle cells, their immediate precursors, and pericytes. We conclude that the increased alpha -sm-actin immunoreactivity represents muscularization of the vessel wall in functional terms and may be regarded as one structural cause among others for the establishment of persistent fetal circulation in hypoplastic lungs.