功能性便秘儿童的肛门直肠动力学特征和治疗的研究

目的研究功能性便秘患儿的直肠肛门动力学改变,观察便秘患儿对根据检测结果制定的相应方案的治疗反应。方法2004年5月至2005年6月采用多功能消化道测压仪观察便秘组即功能性便秘患儿[8例,年龄(11±5)岁]及对照组即排便习惯正常儿童[10例,年龄(11±4)岁]的肛门直肠动力学指标,并进行比较;对便秘组采用相应保守治疗方案,即结合肛门直肠动力学特征、口服双歧三联活菌制剂和益生元类制剂乳果糖(商品名杜秘克)、定时敦促排便,观察其疗效。结果便秘组和对照组的肛门括约肌有效长度、最大肛门括约肌主动收缩压(MSP)、>50%MSP时间比较差异无统计学意义,而便秘组的最低敏感量和最大耐受量大于对照组(t分别为2.178、1.574,P均<0.05);8例便秘患儿保守治疗,7例有效。结论功能性便秘患儿存在肛门直肠动力学异常;以动力学改变为依据的相应保守治疗方案有显著的临床疗效。     

肛门直肠畸形患儿手术后肛门括约肌肛管内超声评价及意义

目的应用肛管内超声检查评价肛门直肠畸形术后患儿肛门内括约肌(internal anal sphincter,IAS)、肛门外括约肌(external anal sphincter,EAS)功能,并与正常儿童的测量结果进行比较,分析括约肌受损程度与肛门功能的相关性。方法回顾性分析山西省儿童医院2014年8月至2016年10月收治的122例肛门直肠畸形手术后患儿的肛管内超声资料及临床资料,同期纳入50例消化系统功能正常儿童作为对照,对所有纳入研究的受试对象进行括约肌受损程度评分;通过Spearman秩相关分析评价括约肌评分和肛门功能评分之间的相关性。结果正常儿童IAS、EAS的厚度与性别、年龄无关(P0. 05);肛门直肠畸形术后患儿IAS、EAS最大厚度与正常儿童比较无明显差异(P 0. 05);不同类型肛门直肠畸形术后患儿IAS、EAS最大厚度无显著差异(P 0. 05);肛门直肠畸形术后患儿括约肌受损程度与肛门功能评分呈负相关(r=-0. 728,P 0. 05)。结论肛门直肠畸形术后患儿肛管内超声结果提示其IAS、EAS最大厚度与正常儿童无明显差别,但存在局部缺损或变薄,其完整性、受损程度与肛门直肠畸形术后排便功能密切相关。     

肠神经发育不良患儿直肠肛门测压与分析

目的分析肠神经发育不良(IND)患儿直肠肛门测压有关参数,探讨其与IND病理改变程度的关系及直肠肛门测压在IND诊断中的作用。方法对20例重型肠神经发育不良(SIND)和41例轻型肠神经发育不良(MIND)患儿及20例功能性便秘(FC)患儿同时作直肠肛门测压检查。结果SIND患儿平均发病年龄明显低于MIND及FC患儿;SIND患儿直肠肛门抑制反射(RAIR)发生率明显低于MIND及FC患儿;与MIND及FC患儿比较,SIND患儿直肠静止压、肛管静止压、肛管高压带长度及直肠顺应性之间无明显差异;但平均反射阈值、平均反射潜伏期及平均反射持续时间之间却有显著差异。IND直肠肛门测压并无特征性改变,RAIR表现为不典型松弛波,呈“W”或“V”形,其反射阈值增大,反射潜伏期及反射持续时间长,RAIR与IND病理改变程度呈相关性变化。结论结合钡灌和乙酰胆碱酯酶组织化学检查,直肠肛门测压可协助诊断IND,并可作为反应IND病理类型的一项参考指标。     

腹腔镜辅助高位肛门直肠畸形成形术后中期随访研究

目的 探讨高位肛门直肠畸形患儿腹腔镜辅助肛门直肠成形术(LAARP)的疗效.方法 回顾分析了2002年1月至2005年12月在我院采用LAARP高位肛门直肠畸形10例,并与2002年1月之前行后矢状入路肛门直肠成形术(PSARP)的12例患儿临床情况进行比较.所有患儿均在新生儿期行结肠造口术.LAARP组在3～5岁、PSARP组在6～8岁时,所有患儿的直肠肛门功能通过Kelly排便功能评分法(6分法)以及肛门直肠测压法进行评价.结果 LAARP组行肛门直肠功能评价的平均年龄为(47.2±8.3)个月,低于PSARP组的(74.5±9.3)个月,差异有统计学意义(P<0.05).LAARP组与PSARP组Kelly排便功能评分分别为3.7±0.9与3.4±0.8;二组肛管静息压、肛管高压带长度十分类似,差异无统计学意义(P>0.05),而二组直肠肛门抑制反射差异有统计学意义(P<0.05).结论 中期随访研究表明,高位肛门直肠畸形患儿LAARP可获得满意的排便控制效果,但LAARP与PSARP对长期生活质量的影响还有待于长期随访的结果.     

肛门直肠及畸形发育胚胎学研究进展

先天性肛门直肠畸形是小儿常见的消化道畸形之一 ,发病率为 1/5 0 0 0～ 1/15 0 0 [1] 。目前认为肛门直肠畸形的发生是胚胎期发育障碍的结果 ,但具体发育过程及畸形演变规律仍不清楚。笔者就近年来肛门直肠及畸形胚胎学研究与进展做一综述。一、肛门直肠正常胚胎发育1.泄殖腔与膈观点　多数学者认为[1] ,胚胎第 3周末 ,后肠末端膨胀与前面的尿囊相交通 ,形成泄殖腔。中肾管 原肾管开口于泄殖腔内。泄殖腔的尾端被外胚层的一层上皮细胞所封闭 ,与体外相隔 ,称为泄殖腔膜。第 4周位于泄殖腔与后肠间的中胚层皱襞形成并向尾侧生长 ,同时间充质…     

新生儿期一期肛门成形术治疗中位肛门直肠畸形疗效评价CSCD

目的总结中位肛门直肠畸形患儿行新生儿期一期肛门成形术与分期肛门成形术的远期排便功能及肛门直肠测压结果,评估新生儿期一期肛门成形术治疗中位肛门直肠畸形的临床疗效。方法回顾性收集2006年1月至2019年8月上海交通大学医学院附属新华医院小儿外科收治的132例中位肛门直肠畸形患儿临床资料,手术方式均为改良后矢状入路肛门成形术(mini-Peña),按照手术时期分为两组,A组为新生儿期接受一期肛门成形术的患儿,共22例;B组为接受传统三期肛门成形术的患儿,共110例。两组最远随访至术后15年。评估两组临床情况以及远期排便功能、并发症情况。术后排便功能评估采用Rintala评分,结果分为优、良、中、差四个等级。肛门直肠测压包括肛门静息压、肛门长度、内括约肌静息压及长度、外括约肌静息压及长度、直肠肛门抑制反射(rectoanal inhibitory reflex,RAIR)等。结果两组患儿性别比例、出生体重差异均无统计学意义(P>0.05)。单次肛门成形术后住院时间比较,A组长于B组[(17.1±3.9)d比(10.4±3.4)d,P<0.05];总住院时间及总手术时间比较,A组均明显短于B组,分别为(19.6±5.5)d比(37.5±10.7)d、(128.9±29.0)min比(287.9±61.2)min,差异均有统计学意义(P<0.05)。两组肛门长度以及内、外括约肌长度差异均无统计学意义(P>0.05);A组肛门静息压、内括约肌静息压及外括约肌静息压均高于B组,分别为(53.8±15.5)mmHg比(36.7±10.4)mmHg、(53.5±15.1)mmHg比(34.6±8.7)mmHg、(45.7±16.9)mmHg比(33.9±11.8)mmHg,差异均有统计学意义(P<0.05)。A组肛门直肠抑制反射引出率明显高于B组(66.7%比0%,P<0.05),其他肛门直肠测压数据差异无统计学意义(P>0.05)。两组Rintala评分优良率差异无统计学意义(83.3%比88.0%,P>0.05)。两组术后便秘、污粪的发生率以及术后并发症的发生率比较,差异均无统计学意义(P>0.05),分别为25.0%比28.0%、16.7%比16.0%、33.3%比50.7%。结论中位肛门直肠畸形行新生儿期一期肛门成形术可以获得良好的肛门控便能力。早期一期肛门成形术可避免造瘘以及造瘘关闭手术的创伤。     

先天性无肛畸形儿MR成像及其与排便功能关系研究

目的评估先天性肛门直肠畸形儿（anorectal malformations，ARM）尾端发育情况及横纹肌复合体（sphincter muscle complex，SMC）发育状态与排便功能的关系。方法39例ARM，男28例，女11例，应用不同序列体部相控阵列线圈或头部线圈MRI显示ARM直肠闭锁水平、骶尾椎、骶髓、泌尿生殖系的发育情况，并对SMC从多个层面进行定量分析，评估其发育状态与临床评分和肛门功能客观检查的关系。结果39例患儿中，MRI T1WI、T2WI扫描判断畸形水平的正确诊断率为100％，T2WI扫描瘘管检出率为96．9％。骶尾椎和骶髓的异常占41．0％，泌尿生殖系统畸形占20．5％。当耻骨直肠肌宽度的相对值PRWR〈0．18，外括约肌宽度的相对值EASWR〈0．15时，71．4％的ARM术后出现肛门失禁。当PRWR〉0．18，EASWR〉0．15时，91．3％的ARM术后排便功能好。结论MR检查能清楚地显示ARM类型、闭锁水平、SMC发育状态、脊柱脊髓及泌尿生殖系统存在的伴发畸形，从MR影像中找到了评价SMC的定量指标。     

新生儿期一期肛门成形术治疗中位肛门直肠畸形疗效评价

目的总结中位肛门直肠畸形患儿行新生儿期一期肛门成形术与分期肛门成形术的远期排便功能及肛门直肠测压结果, 评估新生儿期一期肛门成形术治疗中位肛门直肠畸形的临床疗效。方法回顾性收集2006年1月至2019年8月上海交通大学医学院附属新华医院小儿外科收治的132例中位肛门直肠畸形患儿临床资料, 手术方式均为改良后矢状入路肛门成形术(mini-Pe?a), 按照手术时期分为两组, A组为新生儿期接受一期肛门成形术的患儿, 共22例;B组为接受传统三期肛门成形术的患儿, 共110例。两组最远随访至术后15年。评估两组临床情况以及远期排便功能、并发症情况。术后排便功能评估采用Rintala评分, 结果分为优、良、中、差四个等级。肛门直肠测压包括肛门静息压、肛门长度、内括约肌静息压及长度、外括约肌静息压及长度、直肠肛门抑制反射(rectoanal inhibitory reflex, RAIR)等。结果两组患儿性别比例、出生体重差异均无统计学意义(P>0.05)。单次肛门成形术后住院时间比较, A组长于B组[(17.1±3.9)d比(10.4±3.4)d, P<0.05];总住院时间及总...     

腹腔镜直肠代阴道肛门成形术治疗先天性肛门闭锁直肠前庭瘘并苗勒管发育不全综合征二例

肛门直肠畸形的发生率约为1/3500,其中先天性肛门闭锁前庭瘘是女性肛门直肠畸形中常见的类型。阴道及子宫不发育或发育不良即苗勒管发育不全综合征(Mayer-Rokitanski-Küster-Hauser,MRKH)通常表现为原发性无月经,在出生女婴中发病率约为1/4000~5000。Levitt等[1]报道在肛门直肠畸形前庭瘘的患儿中阴道畸形的发生率为0.6%。同时报道在子宫阴道发育异常的患儿中直肠前庭瘘的发生率为9.5%[2]。Wang等[3]总结病例发现在MRKH中直肠肛门闭锁前庭瘘的发病率为2.25%。我科近年收治了2例先天性肛门闭锁前庭瘘合并MRKH患儿,均一期行肛门成形,直肠前庭瘘代阴道成形术,现报告如下。     

经肛巨结肠根治术后肛肠功能评价

目的探讨经肛门巨结肠根治术后排便模式、结肠功能和肛门括约肌功能。方法对58例经肛门巨结肠根治术后半年以上儿童进行随访,并进行排便功能问卷调查、钡灌肠、结肠传输时间和肛门直肠测压检查,对其术后肛肠功能进行综合评价。结果58例接受随访的患儿中,4例(4/58)稀便、便频,9例(9/58)污便,5例(5/58)便秘,3例(3/58)小肠结肠炎;钡灌肠结果:结肠形态恢复良好,乙状结肠迂曲减少或消失,与切除肠段长短相关;全部病例术后直肠肛管角(°)较对照组显著开大(123.3±15.1vs79.0±11.6,P<0.01),有症状组又较无症状组显著开大(135.6±15.9vs111.0±14.3,P<0.05)。结肠传输时间(h):与对照组比较,有症状组显著缩短(25.2±5.6vs28.7±7.7,P<0.05。直肠肛管反射3例阳性,2例弱阳性;肛管最大压力(mmHg):便秘组的最大收缩压明显高于无症状组和对照组(211±36vs200±65,P<0.05;211±36vs190±38,P<0.05);向量容积(cm×cmHg2)和对称指数:污便组向量容积和对称指数较术前及对照组显著降低(381±109vs662±31,P<0.05;0.69±0.32vs0.70±0.07,P<0.05);便秘组对称指数与术前无显著差异,但显著高于对照组(0.74±0.02vs0.70±0.07,P<0.05)。结论经肛门巨结肠根治术后排便功能、结肠功能和括约肌功能多数良好。少数病例排便功能障碍可能与术后乙状结肠曲减少或消失、“新直肠”储便功能代偿不全和拖出结肠致直肠肛管角开大、肛门括约肌痉挛失迟缓有关。     

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.