腹腔镜辅助小切口在新生儿长段型巨结肠根治术中的应用

目的探讨新生儿长段型先天性巨结肠的微创手术方法。方法采用腹腔镜辅助经脐小切口拖出病变肠管,于直视下处理肠系膜,经肛门Soave术一期根治新生儿长段型先天性巨结肠。结果9例患儿均一期完成手术,平均手术时间100～120min,切除病变肠管26～40cm。其中3例行升结肠翻转术。全部病例于术后第3d进食,第10d扩肛后出院,脐部切口隐蔽,无明显疤痕。结论腹腔镜辅助经脐小切口根治新生儿长段型先天性巨结肠,具有创伤小,术后并发症少,切口小而隐蔽等优点。     

单孔腹腔镜Deloyers术在先天性长段型巨结肠一期根治术中的应用

目的总结单孔腹腔镜Deloyers术在先天性长段型巨结肠手术中的应用经验及技巧。方法回顾性分析2018年1月至2021年5月广州市妇女儿童医疗中心胃肠外科单孔腹腔镜Deloyers术治疗的16例先天性长段型巨结肠患儿的临床资料, 其中男11例, 女5例;年龄为3～10个月;体重为5.0～12.8 kg;均行升结肠倒转, 评价其手术效果, 总结单孔腹腔镜下Deloyers术的经验。结果 16例患儿均一期完成单孔腹腔镜下巨结肠根治术(Deloyers倒转法), 术中完成单孔腹腔镜下结肠韧带松解及乙状结肠、直肠游离后, 将结肠经脐部提出腹腔外直视下完成结肠系膜分离及升结肠倒转, 无中转开放或需增加Trocar病例。手术时间为(217±30)min, 出血量为5(2～10)ml, 术后住院时间为7(5～8)d。术后均恢复良好, 随访6～46个月, 无出血、肠梗阻、吻合口漏、便秘复发、大便失禁等并发症, 术后6个月后患儿排便次数为2～5次/d, 术后腹部均无可视性瘢痕。结论单孔腹腔镜Deloyers术可应用于先天性长段型巨结肠的一期根治术, 具有操作精准、便捷、创伤小、美观等优点, 术后短期效果...     

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。     

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.     

经肛门拖出及辅助腹部小切口根治长段型巨结肠33例报告

目的探讨经肛门拖出及/或选择性辅加腹部小切口根治长段型巨结肠的可行性与疗效。方法2001年1月～2005年3月经用该术式治疗婴儿长段型巨结肠33例。其中病变段位于乙状结肠上段16例,降结肠11例,脾曲4例,横结肠右侧2例。全组病例均经钡剂灌肠、肛门直肠测压、手术及病理检查确诊。实施I期手术32例,II期手术1例。结果17例单独经肛门拖出结肠,16例辅加腹部3～5cm小切口协助完成手术。切除病变结肠平均长度为39.2cm(32～63cm)。平均手术时间为145min(110～190min)。出血约10～50ml。术后恢复良好,6例出现肛周轻度红肿、糜烂,均于术后6～11d痊愈出院。30例经3个月～4.5年随访,生长发育良好,3个月内每日排便4～8次,6个月后每日2～3次。5例发生结肠炎,经保守治疗痊愈。轻度污粪及便秘各1例。无腹部并发症及吻合口狭窄。结论经肛门结肠拖出术根治婴儿长段型巨结肠方法可行,且安全、有效,操作较简便。对病变位于降结肠以上、结肠系膜较短者,可辅加腹部小切口协助完成手术,值得推荐。     

腹腔镜与开腹手术治疗新生儿长段型巨结肠的对比研究

目的 探讨腹腔镜与开腹手术治疗新生儿长段型巨结肠的近期疗效.方法 2007年1月至2009年1月作者收治新生儿长段型巨结肠17例,均表现为顽固性便秘,钡灌肠24 h拍片均见大量钡剂残留.8例采取腹腔镜手术,其中7例直肠肛管测压无松弛反射,直肠黏膜乙酰胆碱酯酶染色均为阳性;9例采取开腹手术,7例无松弛反射,直肠黏膜乙酰胆碱酯酶染色均为阳性.结果 17例中,5例行左半结肠切除术,12例行右半结肠切除术.均治愈,并发切口裂开1例,吻合口瘘1例,为开腹手术患儿.随访左半结肠切除患儿术后大便形状正常;结肠次全切除患儿术后1个月左有出现腹泻,后逐渐好转;随访期间3例发生小肠结肠炎,经抗炎、洗肠等治疗后痊愈,其中1例为腹腔镜手术,2例为开腹手术.结论 腹腔镜巨结肠根治术治疗新生儿长段型巨结肠可取得满意的近期疗效.     

Ⅰ期经肛门巨结肠根治术15例报告

目的 报告一种新型I期肛门巨结肠根治术及期手术效果。方法 15例经组织学检查诊断为直肠乙状结肠型巨结肠患儿均行I期经肛门巨结肠根治术,方法为在直肠后壁齿状线上1cm、前壁齿状线上2－3cm切开直肠粘膜,向近端游离达腹腔水平后,切除腹膜外直肠肌鞘至肛提肌水平,残留肌鞘后壁“V”形切除。游离近端结肠,拖出正常结肠与肛门斜行吻合。结果 平均手术时间145min(95-250min）,平均出血45ml。无术中和术后并发症。随访1－4个月所有患儿排便2－6次／d,无污粪。结论 I期经肛门巨结肠根治术安全有效,远期效果有待随访。     

儿童腹腔镜下巨结肠根治、结直肠钉合吻合术的应用

目的评价腹腔镜辅助下结直肠切除、结直肠钉合吻合术治疗儿童先天性巨结肠的疗效。方法2010年2月至2012年2月我们对12例4—14岁常见型先天性巨结肠患儿,采取腹腔镜辅助下病变肠段游离经肛门拖出切除（改良Swenson手术）、结直肠采用吻合器完成钉合吻合术,统计手术时间、出血量、住院时间、住院期间排便情况和近期并发症。结果12例患儿均在腹腔镜辅助下顺利完成改良Swenson手术、结直肠钉合吻合术,手术时间1．5～2．0h;术中出血3～5mL;无术中并发症及术后伤口感染。2例术后出现排便时肛门疼痛;2例出现吻合口瘘,1例保守治疗30d瘘口愈合;1例行横结肠造瘘,术后4个月出现结直肠吻合口狭窄,经肛门纵行切开横行缝合后扩肛缓解;1例出现肠炎症状给予对症治疗痊愈。7例获随访患儿均无污粪、便秘情况存在,每日排便1～3次。所有患儿小便正常。结论腹腔镜辅助巨结肠根治、结直肠吻合器钉合方法可行,手术时间明显缩短,出血少,创伤小,术后恢复快,短期随访疗效满意,适合儿童巨结肠手术,但婴幼儿不适合此法。     

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。     

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.     

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   

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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     

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.     

A profile of respiratory symptoms in urban and rural South Australian school children

This report describes the cross-sectional analyses of data from the first year of a longitudinal study using questionnaire and respiratory function data over a 5 year period from a sample of rural South Australian school children. The cumulative or lifetime prevalences of respiratory symptoms were estimated in 825 rural and 1261 urban school children aged between 5 and 15 years in order to determine if the prevalence rates differed between rural and urban school children. The study found the overall cumulative prevalence of asthma and/or wheezy breathing (AWB) to be 24.1% in the rural school children compared to 27.6% in the urban school children. Most children developed AWB symptoms before the age of 7 years, with 20% reporting moderately severe symptoms and 10% having more than one attack per fortnight. The cumulative prevalence of bronchitis, loose/rattly cough (BLRC) differed significantly between the rural school children (34.1%) and urban school children (47.9%). The BLRC symptoms preceded the development of AWB in many cases. Urban school children also reported a higher prevalence of atopic conditions.     

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.     

Hauttemperaturen verschiedener Körperoberflächenareale des Rumpfes bei Säuglingen

Summary In two groups of infants (3–53 weeks old) skin temperatures were controlled in different areas of the trunk—i.e.: regions of sternum, lungs, heart, liver, spleen, kidneys—at different room-temperatures (group I: 21–25°C; group II: 29–32°C). Rectal temperatures of some probands in both groups also had been controlled simultaneously. A definite change in the reaction to heat was proofed in different periods of the first year of life. In higher environmental temperatures the skin temperature was almost constant at every controll-point of the skin, even in older infants. In lower environmental temperatures the skin temperatures lowered continuously with age till 7. to 9. moth. From 10. to 12. month the lowering of skin temperature discontinued. The rectal temperatures were relatively constant in all infants. Only in infants from 7. to 12. month, whose skin temperatures were controlled in lower as well as in higher environmental temperatures, a tendency to higher rectal temperatures was proofed in warmer environmental temperatures.The significance of these results is discussed.

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Untersuchungen mit Unterstützung durch die Deutsche Forschungsgemeinschaft.