先天性巨结肠小肠结肠炎肠道乳酸菌群的检测

目的通过对巨结肠小肠结肠炎、无肠炎患儿及正常儿童大便中双歧杆菌和乳酸杆菌进行检测，初步探讨乳酸菌群和先天性巨结肠小肠结肠炎的关系。方法选取2006年1月至2006年12月在上海交通大学医学院附属新华医院行先天性巨结肠根治术的患儿共30例，1个月～7岁，平均年龄1、2岁。根据术前术后有无小肠结肠炎临床症状分为HD小肠结肠炎组和HD非肠炎组．其中肠炎10例。并选同龄儿10例为正常对照组。分别采集各组的新鲜大便，立即置入无菌离心管．-20℃冻存，24h内抽提粪便内细菌基因组DNA。结果由于粪便内双歧杆菌和乳酸杆菌绝对值不呈正态分布，故取其对数值（log10）进行方差分析。各组双歧杆菌对数含量：巨结肠肠炎组7．07±0．85和巨结肠非肠炎组8．36±0．81）、正常对照组8.46±0．92比较．差异均有统计学意义（P〈0．05）。巨结肠非肠炎组和正常对照组相似，差异无统计学意义（P〉0．05）。各组乳酸杆菌对数含量：巨结肠肠炎组5．51±0．65、巨结肠非肠炎组5．79±0．82，分别和正常对照组6．47±0．71比较，差异有统计学意义（P〈0．05）。结论结合本实验结果提示在巨结肠患儿中双歧杆菌和乳酸杆菌的含量明显低于正常组，这可能是肠炎发生的原因之一。     

先天性巨结肠术后肠炎的结肠镜下特征分析

目的 探讨小儿先天性巨结肠(HD)术后小肠结肠炎(EC)的结肠镜下表现及其与手术因素的关系.方法 我院238例HD术后患儿随访1～8年,其中临床诊断为EC者45例,均行结肠镜检查.观察EC的结肠镜下表现,并将EC组与非EC组其他手术并发症的发生率进行比较.结果 肠镜观察EC,近吻合口结肠表现为黏膜水肿质碎、充血、大片糜烂,9例合并溃疡;远离吻合口结肠主要表现为黏膜充血水肿,点片状糜烂,3例合并溃疡;吻合口周围结肠炎症较回肓部重(P＜0.01).EC组其他手术并发症17例(吻合口病变8例,复发3例,解剖异常6例),非EC组13例(吻合口病变7例,复发1例,解剖异常3例);EC组手术并发症发生率37.8％高于非EC组6.7％(P＜0.01).结论 HD合并手术并发症者,EC发生率高;可能因手术并发症引起病变主要集中在吻合口及周围结肠,肠镜观察EC表现为近吻合口周围结肠炎症较重,远离吻合口逐渐减轻.     

腹腔镜辅助结肠次全切除术治疗先天性肠神经元发育不良症的对比研究

目的：对比研究先天性肠神经元发育不良症B型患儿行腹腔镜下结肠次全切除术、Soave术和Duhamel术的临床疗效。方法回顾性分析2005年3月至2013年3月接受腹腔镜结肠次全切除的B型肠神经元发育不良患儿的随访资料。其中接受腹腔镜辅助下Duhamel术（直肠后拖出术）28例,同期接受腹腔镜辅助下Soave术（直肠内拖出术）34例。入选的62例患儿诊断依据为临床表现、术前影像学结果、直肠黏膜活检、术中快检和术后病检报告。分别比较两组手术时间、术中出血量、术后住院时间、排便频率、术后并发症及排便功能方面有无差异。结果腹腔镜Duhamel术和腹腔镜Soave术在手术时间、术中出血量、术后住院时间方面比较,均无统计学意义（P＞0.05）。排便频率比较：腹腔镜下Duhamel术患儿术后2周内排便频率平均8次/d,明显少于腹腔镜下Soave术的15次（P＜0.05）;恢复到正常排便频率（1～2次/d）时间比较：腹腔镜下Duhamel术平均为（3.5±0.5）个月,腹腔镜下Soave术平均为（6.7±0.8）个月,差异有统计学意义（P＜0.05）。腹腔镜下Duhamel术患儿术后发生小肠结肠炎1例（3.6％）,污粪1例（3.6％）,粪石1例（3.6％）;腹腔镜下Soave术后发生小肠结肠炎4例（11.8％）,吻合口狭窄1例（2.9％）,便秘复发2例（5.8％）,污粪1例（2.9％）。肛周湿疹的发生率：Soave术组明显高于Duhamel术组（41.2％VS 7.2％）。58例术后获功能随访,平均随访时间为4年6个月（2个月至8年）。接受腹腔镜下Duhamel术的患儿其排便功能优良率为85.2％,腹腔镜下Soave术为87.0％。两组差异无统计学意义（P＞0.05）。结论行结肠次全切除的B型肠神经元发育不良患儿,腹腔镜下Duhamel术后小肠结肠炎的发生率低于Soave术式,腹腔镜下Duhamel术后近期排便频率少,且排便频率更快恢复正常,肛周湿疹发生率低。     

先天性巨结肠及同源病患儿术后直肠肛管测压与分析

目的分析先天性巨结肠（HD）及同源病（HAD）术后患儿直肠肛管测压参数及其临床意义。方法对56例巨结肠术后患儿，包括32例HD和24例HAD，进行随访，并根据排便功能分为正常组、污粪组及便秘组。同时进行直肠肛管测压检查。根据不同病理分型，临床症状及随访时间比较分析测压结果。结果直肠肛管抑制反射（RAIR）重现率为55％，术后1a内RAIR重现率明显低于1a以上患儿（P〈0．05）。HD术后患儿肛管直肠蠕动频率明显大于HAD（P〈0．05）。按主观症状将患儿分为正常、污粪和便秘组，便秘组肛管／直肠静息压之比及高压带长度与前二组比较有显著差异（Pa〈0．01）。结论部分患儿术后RAIR可恢复，并与术后时间有关，HD术后肛门功能改善优于HAD。影响术后测压结果的因素是多方面的。     

巨结肠根治术后小肠结肠炎：有关高危因素分析

１９８２～１９９２年采用根治术治疗先天性巨结肠１０３例，其中１６例（６．４３％）发生术后小肠结肠炎。分析有关因素，发现性别、手术时年龄、无神经节细胞肠段长度及根治术类型等对其发生影响不大，但与吻合口狭窄及术前小肠结肠炎的发生、肠炎发作与再根治术之间间隔的时间密切有关。观察术中拖出结肠段的病理组织学改变，提示拖出肠段明显炎性改变是发生术后小肠结肠炎的病理基础，并对有关因素进行了讨论。     

先天性巨结肠Swenson改良术后排便功能与生活质量研究

目的：评估先天性巨结肠Swenson改良术后排便功能和生活质量。方法：随访常见型先天性巨结肠Swenson行改良术后患儿45例（男37例，女8例），手术年龄2．5个月－6岁，平均1．6岁。术后随访8－16年，平均10．9年，评估其排便功能和生活质量。结果：术后并发症有：污染和小肠结肠炎17例（37．8％），便秘3例（6．7％），大便失禁3例（6．7％），小便失禁4例（8．9％）；吻合口狭窄2例（4．4％）和粘连性梗阻1例（2．2％）。23例（51％）表现不同程度的排便障碍，但肛门功能优良率仍达到84．4％（李正评分）和93．4％（Rintala评分），生活质量评分显示，优者18例（40％），良者21例（46．7％），差者6例（13．3％），结论：先天性巨结肠Swenson改良术后，多数患儿远期肛门功能和生活质量优良，排便功能障碍是影响生活质量的主要因素。     

快速康复外科技术在1-4月龄婴儿巨结肠根治术中的应用

目的探讨快速康复外科技术（FTS）应用于小婴儿巨结肠中的可行性、安全性及临床与社会价值。方法收集本院2010年10月至2013年10月应用快速康复外科技术治疗的1-4月龄婴儿巨结肠53例,为 FTS 组;选取2008年10月至2010年10月采取传统方式治疗,与 FTS 组条件相同,总体临床特点相似的1-4月龄婴儿巨结肠40例,作为对照组。全部病例均进行一期巨结肠根治术,并获随访,随访时间6个月至5．5年;对两组病例术后首次排便时间、停止输液时间、住院时间、治疗费用和中期并发症等方面进行比较。结果FTS 组与传统组术后停止输液时间分别是（2．4±1．1）d、（3．5±1．2）d;住院时间分别是（7．4±1．2）d、（10．5±1．6）d;治疗费用分别是（8300±840）元、（11100±920）元,差异均有统计学意义（P ＜0．05）。两组术后首次排便时间分别是（10．3±1．8）h 和（11．5±2.4）h,第3天 CRP 正常比例分别是73．6％和55％,差异无统计学意义（P ＞0．05）;两组术后中期效果指标（便秘、污粪／肛门失禁、肛周感染,小肠结肠炎）比较,无显著差异（P ＞0．05）。结论快速康复外科技术应用于1-4月龄婴儿巨结肠可明显加速患儿康复,缩短住院时间,节省住院费用,安全有效,但需要逐步推进和多部门密切配合。     

先天性巨结肠根治术后小肠结肠炎188例分析

目的 探讨先天性巨结肠根治术后小肠结肠炎发生的相关性,分析术后小肠结肠炎的高危因素.方法 收集2009 ～2012年先天性巨结肠及同源病根治手术治疗病例188例,根据病理诊断与手术切除范围不同分组,对比各组间术后小肠结肠炎发生率,分析术后小肠结肠炎发生的相关性.结果 先天性巨结肠与巨结肠同源病在术前小肠结肠炎发病率无明显差异（P＞0.05）,术后巨结肠同源病发生小肠结肠炎的几率明显高于先天性巨结肠（P＜0.05）;术前发生小肠结肠炎与术后发生小肠结肠炎明显相关（P＜0.05）;术中结肠切除范围和术后小肠结肠炎之间无明显相关（P＞0.05）;Soave与心形吻合方式对于术后小肠结肠炎的发生无明显相关（P＞0.05）;全结肠型巨结肠更易发小肠结肠炎.结论 术前小肠结肠炎、巨结肠同源病及全结肠型巨结肠仍然是影响术后小肠结肠炎发生的重要因素.     

结肠-直肠心形吻合术治疗先天性巨结肠远期疗效分析

目的：分析我院1992－2001年采用直肠肛管纵结肠－直肠心形吻合术治疗先天性巨结肠（HD）并获长期随访的136例远期疗效。方法：本组136例，其中新生儿40例，13例出现巨结肠危害。常见型96例，长段型18例，短段型22例，136例均实施Ⅰ期根治心形吻合术，术后随访18个月－10年，随访内容包括临床主观排便功能检查和客观排便功能测定等。结果：无手术死亡，21人近期并发小肠结肠炎（17．6％），远期随访排便正常127例，异常9例，便秘2例（1．5％），污粪7例（5．1％），直肠肛管测压84人次（84／136），直肠肛管抑制反复恢复46例，占54．8％（46／84），结论：直肠肛管纵切心形吻合术适用于各年龄组及绝大多数类型巨结肠的根治，无神经节细胞肠管长度以及根治时的年龄是影响远期肛门功能的重要因素。     

先天性巨结肠经肛门Soave Ⅰ期拖出术后功能随访

目的评估先天性巨结肠经肛门Soave Ⅰ期拖出术后排便功能。方法随访2000年10月-2004年10月经肛门Ⅰ期拖出术44例，开腹Soave术35例，腹腔镜Soave术29例，Ikeda-soper术39例。术后随访1～5年。对围手术期疗效（术前准备时间、手术时间、术中输血量、术后禁食天数、应用抗生素时间、住院天数及费用）及术后排便控制能力（Kelly评分和直肠肛管测压）进行评估。结果经肛门Ⅰ期拖出术平均手术时间（75±20）min、费用（8198．81元）低于开腹术[（92±25）min；10264．45元]、腹腔镜术[（125±25）min；12504．67元]（P〈0．05）；术前肠道准备（2d）、手术时间（75±20）min、术中输血量（0例）、术后禁食天数（2d）、应用抗生素时间（4±1）d、住院天数（11±2）d及费用（8198．81）元较Ikeda-soper术[（4±1）d；（240±30）min；32例；（5±1）d；（7±2）d；（19±3）d；15243．78元]显著降低（P〈0．01）。经肛门Ⅰ期拖出术后的近远期并发症发生情况与开腹术、腹腔镜术无显著差别（P〉0．05）；Soave术后3个月小肠结肠炎的发生率（12例）高于Ikeda-soper术（3例）（P〈0．05），但就经肛门Soave术（5例）与Ikeda-soper术（3例）比较，差异无显著性意义（P〉0．05）。经肛门Ⅰ期拖出术和Ikeda-soper术患儿术后12个月Kelly评分无差别；术后1年直肠肛管测压均未引出直肠肛管抑制反射，术后肛管高压区长度、肛管静息压、直肠感觉阈值无统计学差异，但经肛门SoaveⅠ期拖出术后主动收缩压（52．3±15．6）mmHg低于Ikeda-soper术后（55．7±15．4）ramHg。结论经肛门SoaveⅠ期拖出术创伤小、并发症少、费用明显低于开腹、腹腔镜Soave术以及Ikeda-soper术，适用于治疗小年龄婴幼儿的短段型和常见型先天性巨结肠。     

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     

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.     

How well are we doing? – Metabolic control in patients with diabetes

OBJECTIVE: To compare the present level of metabolic control in children and adolescents with insulin-dependent diabetes mellitus (IDDM) attending Brisbane paediatric diabetes clinics with published overseas data. METHODOLOGY: Blood HbA1c concentrations, population characteristics, current treatment practices and short-term complications were recorded in all patients, aged 19 years and under, attending the diabetes clinics of the two Brisbane Children's Hospitals or the private practice of one of the authors (MJT) in the first quarter of 1998. RESULTS: Two hundred and sixty-eight patients were assessed (M/F 142/126). Ages ranged from 1 to 19 years (mean 11. 2 years); duration of IDDM was 0-16 years (mean 4.4 years); and 141 (53%) were pubertal. Of those aged less than 13 years, only 4% had more than two injections daily. Insulin doses (U/kg/day) rose with increasing age. Larger doses were required in regimens involving more than two injections per day than those involving one to two injections per day. Ketoacidosis or severe hypoglycaemia in the last 3 months were reported in eight (2.7%) and 17 (6.3%) of patients, respectively. Mean HbA1c (+/- SD) was 8.6 +/- 1.4% (range 5.2-14.0%), with 33% of children having a HbA1c concentration < 8%. HbA1c concentrations were significantly related (P < 0.05) to insulin dose and to duration of diabetes, but not to severe hypoglycaemia, ketoacidosis, age, frequency of injections, or number of clinic visits per year. Mean HbA1c concentration was significantly higher (P < 0.05) in those children in puberty (8.7 +/- 1.5%) than in those not in puberty (8.5 +/- 1.2%). CONCLUSION: Only 33% of patients had a HbA1C concentration less than 8% and 6.3% had a severe hypoglycaemic episode in the 3 months. These results are similar to published overseas data.