经肛门改良Soave Ⅰ期拖出术治疗小儿先天性巨结肠症

目的总结经肛门改良SoaveⅠ期拖出术治疗小儿先天性巨结肠症的疗效及随访情况。方法自2001年采用该术式治疗小儿先天性巨结肠症26例，术前均经钡灌肠、直肠粘膜吸引活检等辅助检查诊断为先天性巨结肠症。结果切除结肠16cm-22cm，术后24h内肠道功能恢复，第2～3d开始进食无异常。术后1月随访，每日排便6-9次，其中12例行钡灌肠检查，未发现扩张结肠，24h钡剂无残留；18例6个月随访，每日排便2-4次；17例1年随访，5例诉时有大便次数多(每日排便5～6次)，12患儿每日排便2～3次；14例2年随访，1例因不洁饮食腹泻后出现腹胀1次，补液后缓解，13例排便正常，每日1-3次；12例术后已3年，生长发育正常，每日排便1-3次。全部患儿未行扩肛处理，无腹胀、便秘、污粪。结论经肛门改良SoaveI期拖出术治疗小儿常见型先天性巨结肠症，手术创伤小，操作简单，近期疗效好。     

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。     

小婴儿I期经肛门巨结肠根治术

目的研究分析I期经肛门治疗小婴儿先天性巨结肠的临床效果。方法12例患先天性巨结肠小婴儿,11例I期经肛门完成根治术,手术年龄32d～158d。手术采用截石位或俯卧位,于直肠后壁齿状线上0.5cm～1.0cm,前壁齿状线上1.5cm～3.0cm切开直肠粘膜,向近端游离直肠粘膜达腹膜返折,环形切开肌鞘,游离近端结肠,拖出正常结肠与肛门斜形吻合。结果平均手术时间95min,均于12h内排便,无术中术后并发症。随访2个月～1年,临床效果良,无污便。结论I期经肛门巨结肠根治术安全简单,临床效果好,适于在小婴儿中开展。     

改良Soave术治疗婴儿和新生儿先天性巨结肠27例

目的总结改良Soave根治术治疗婴儿和新生儿先天性巨结肠的手术体会。方法对27例10d￣3个月的先天性巨结肠患儿行改良Soave根治术,其中14例常见型巨结肠经肛门直接拖出,4例因根治术前肠穿孔行结肠造瘘术。腹部不切开肠管亦不剥离肌鞘,而是转至会阴部操作,保留肌鞘后壁距齿状线0.5cm,前壁距齿状线2￣3cm。结果术后无内括约肌征候群及肌鞘感染,每月随访1次,无便秘、腹胀及失禁现象,大便控制良好。结论改良Soave根治术应用于小婴儿和新生儿先天性巨结肠,创伤小,恢复快,减轻了患儿痛苦,术后可获得良好的排便控制功能,近期疗效满意。     

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。     

先天性巨结肠50例

目的　探讨小儿先天性巨结肠的临床特点。方法　术前回流灌肠 ,34例行改良Duhamel 环钳斜吻合术 ,14例行经直肠拖出巨结肠根治术 ,2例行强力扩肛术。结果　 5 0例患儿中肛门便意感好 ,能控制排便 4 5例 ;4例肛门有便意 ,白天控制排便 ,夜间偶有污粪 ;1例死亡。结论　先天性巨结肠是新生儿常见的畸形 ,及早手术治疗 ,提高围术期处理 ,对患儿生长发育至关重要     

小婴儿Ⅰ期经肛门巨结肠根治术

目的 研究分析Ⅰ期经肛门治疗小婴儿先天性巨结肠的临床效果。方法 12例患先天性巨结肠小婴儿，11例Ⅰ期经肛门完成根治术，手术年龄32d～158d。手术采用截石位或俯卧位，于直肠后壁齿状线上0．5cm～1．0cm，前壁齿状线上1．5cm～3．0cm切开直肠粘膜，向近端游离直肠粘膜达腹膜返折，环形切开肌鞘，游离近端结肠，拖出正常结肠与肛门斜形吻合。结果 平均手术时间95min，均于12h内排便，无术中术后并发症。随访2个月—1年，临床效果良，无污便。结论 Ⅰ期经肛门巨结肠根治术安全简单，临床效果好，适于在小婴儿中开展。     

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。     

经肛门结肠拖出术(Soave术)治疗新生儿先天性巨结肠

目的 报道采用微创方法治疗新生儿先天性巨结肠。方法 自2001年10月起采用经肛门结肠拖出术(Soave术)治疗新生儿先天性巨结肠35例。结果 35例Ⅰ期手术均成功，术后2d拔除胃管开始进食，14d开始扩肛。随访6个月，无1例出现污粪、失禁、便秘等早期并发症。结论 经肛门结肠拖出术治疗新生儿先天性巨结肠，手术操作简单，创伤小，近期疗效良好。     

116例疑诊新生儿先天性巨结肠的诊治分析

目的探讨新生儿期疑诊先天性巨结肠的诊断与治疗选择。方法回顾性分析2010年12月至2014年3月我们收治的116例新生儿期疑诊为先天性巨结肠患儿的临床资料。其中10例确诊为先天性巨结肠后于新生儿期行巨结肠根治术;94例先予清洁回流灌肠,症状缓解者,教会家长清洁回流灌肠或扩肛等保守治疗措施后出院,3个月后复查,其中28例排便正常,获痊愈,60例症状未缓解者经确诊后行巨结肠根治术,6例失随访;12例新生儿期清洁回流灌肠效果不佳,一期行肠造瘘术,二期行巨结肠根治术。结果116例疑诊先天性巨结肠患儿中,随访110例,随访率为94.8%,获随访病例中,82例经手术治疗痊愈,28例经保守治疗痊愈;12例大便次数多,伴肛周糜烂,2例排便困难,1例肛门狭窄,1例偶有污粪。结论对新生儿期疑诊为先天性巨结肠患儿,可先行清洁回流灌肠、扩肛等保守治疗,3个月后明确诊断者再择期手术治疗。对长段型或全结肠型巨结肠经回流灌肠等治疗效果不佳者,可先行一期肠造瘘术,二期行巨结肠根治术。     

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.     

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.