超声测量膀胱壁厚度预测脊柱裂患儿上尿路损害

目的 通过超声测定膀胱壁厚度和尿动力学检查测定膀胱功能,评价隐形脊柱裂患儿膀胱厚度和功能及上尿路损害的相关性,探讨用膀胱壁厚度评估隐性脊柱裂患儿上尿路损害的可能性.方法 选取超声检查确诊上尿路扩张的隐性脊柱裂患儿22例,年龄(8.8±4.9)岁,并选择同期超声检查无上尿路扩张的隐性脊柱裂患儿29例作为对照组,年龄(9.3±5.3)岁.所有患儿均行尿动力学检查,记录最大膀胱容量,充盈期最大逼尿肌压力,逼尿肌漏尿点压和逼尿肌过度活动最高压力.在膀胱充盈至预测正常膀胱容量的60%时行超声检查测量逼尿肌厚度.同时根据超声检查是否扩张将患儿分为有和无上尿路损害组,比较两组膀胱壁厚度的差异,并分析膀胱厚度与尿动力学参数相关性,计算膀胱壁厚度预测上尿路损害统计学指标.结果 上尿路损害组平均膀胱壁厚度(3.4±0.25)mm,显著高于无上尿路损害组的(2.5±0.45)mm,差异有统计学意义(P＜0.05).膀胱壁厚度与逼尿肌过度活动最高压力、逼尿肌漏尿点压和充盈期最大逼尿肌压力均呈正相关(r=0.87、0.91和0.85,P＜0.0001,P＜0.0001和P=0.017).膀胱壁厚度≥3.0 mm预测上尿路损害的灵敏度为90.9%,特异性为79.4%,阳性预测值76.9%,阴性预测值为92.0%.受试者工作特征曲线(ROC)显示超声测量膀胱壁厚度能高度预测隐形脊柱裂患儿上尿路损害的发生,曲线下面积(AUC)为0.929.结论 超声测定隐形脊柱裂患儿膀胱壁厚度可以帮助预测上尿路损害,膀胱壁厚度大于3.0 mm提示隐性脊柱裂患儿上尿路损害可能性大.     

重视小儿排尿功能障碍的诊治

小儿排尿功能障碍多见。常见临床表现有尿频、尿急、尿痛、尿失禁、排尿困难和遗尿等。正确诊断是成功有效治疗的基础。尿动力学检查的普及显著提高了诊断和治疗水平,已经成为诊断排尿障碍的常用工具。小儿首选无创尿动力学检查包括排尿日记、尿流率和残余尿测定及排尿方式观察等。有创尿动力学检查包括膀胱尿道置管测压和影像尿动力学检查。引起排尿异常的常见病有排尿功能发育延迟、泌尿系感染、神经源性膀胱、膀胱过度活动症(OAB)、遗尿症等。尿动力学检查有助于理解发病机制和分类从而科学制定治疗方案。排尿功能障碍除了影响生活质量外,常引起逼尿肌和上尿路继发损害,甚至危及生命。因此,应重视小儿排尿功能障碍的诊治。     

儿童精神性尿频的尿动力学变化

目的 探讨儿童精神性尿频的病因、病理生理变化及其治疗。方法 本组38例，应用尿流动力仪分别记录排尿量、尿流曲线、膀胱压力容积及压力－流率－肌电图。结果 38例中，4例尿动力学安全正常；34例出现膀胱功能异常，占89.5%(34/38)，其中逼尿肌不稳定性收缩者12例，低顺应性膀胱者6例，低顺应性膀胱合并逼尿肌不稳定性收缩者16例；最大膀胱测量容量百分数下降14例。排尿期异常仅5.3%(2/38)，为尿道括约肌过度活跃。尿动力学检测后，84.2%的患儿症状完全消失或好转。结论 逼尿肌不稳定性收缩是最主要的病理生理学改变；排尿训练是主要治疗措施。     

原发性夜遗尿症尿动力学检查评估

目的探讨原发性夜遗尿(PNE)儿童的尿动力学表现形式并评估其价值。方法156例PNE患儿分单症状性遗尿(MPE)(120例)和复杂性遗尿(CPE)(36例)二组。因上尿路疾病需要手术治疗而下尿路功能正常的20例患儿作对照组,进行膀胱压力容积、压力流率和静态尿道压力分布测定。结果MPE组中,逼尿肌不稳定收缩占56.7%(68/120)例,膀胱顺应性下降占3.3%(4/120)例,最大膀胱容量/正常膀胱容量≤80%9例;CPE组中,逼尿肌不稳定收缩占80.6%(29/36)例,膀胱顺应性降低占22.2%(8/36)例,最大膀胱容量/正常膀胱容量≤80%12例,二组比较差异有显著性意义(P<0.01)。MPE组中,尿道高压66例,逼尿肌括约肌协同失调78例;CPE组中,尿道高压25例,逼尿肌括约肌协同失调21例,二组比较差异无显著性意义(P>0.05)。MPE,CPE中逼尿肌不稳定收缩、逼尿肌括约肌协同失调和尿道压增高的发生率高于对照组,而CPE中顺应性下降的发生率显著高于对照组。结论尿动力学检查结果提示MPE、CPE二组遗尿患儿尿动力学检查的必要性。     

遗尿患儿初始尿意与强烈尿意时尿流率及残余尿测定分析

目的分析遗尿患儿在初始尿意和强烈尿意两种不同情况下尿流率和残余尿测定结果的差异,为临床应用尿流率测定来判断遗尿患儿膀胱功能提供参考。方法对来本院就诊的102例原发性遗尿症患儿(男性60例,女性42例),在初始尿意和强烈尿意时分别进行尿流率和残余尿测定,比较两种情况下尿流率参数(排尿量、排尿时间、最大尿流率)及每次残余尿量检查结果。结果初始尿意时测得患儿最大尿流率为(15.5±8.2)m L/s,残余尿量为(2.9±5.9)m L;强烈尿意时测得患儿最大尿流率为(19.9±9.7)m L/s,残余尿量为(5.2±6.9)m L;初始尿意与强烈尿意下最大尿流率和残余尿比较,差异均有统计学意义(P0.05)。最大尿流率随尿量的增加而增加,但当尿量增加到一定程度后最大尿流率反而有下降趋势。男性与女性在初始尿意时的最大尿流率和残余尿没有显著差异,但在强烈尿意时,男性的最大尿流率显著低于女性,(18.5±8.2)m L/s vs(24.2±12.5)m L/s(P0.05),而残余尿量没有差异。结论遗尿患儿在初始尿意和强烈尿意下尿流率-残余尿测定结果有显著差异,遗尿患儿进行自由尿流率测定时不宜过度憋尿,临床上分析尿流率及残余尿测定结果时要考虑尿意状态和排尿量对尿流测定参数的影响。     

肛门直肠畸形术后排尿功能障碍的评价和治疗

目的 评价肛门直肠畸形术后排尿功能障碍的原因及治疗对策.方法 肛门直肠畸形术后患儿10例,男7例,女3例,年龄1～12岁.肛门闭锁直肠尿道球部瘘4例,肛门闭锁直肠尿道前列腺部瘘3例,泄殖腔畸形1例(共同管＜3 cm),肛门闭锁并球形结肠1例,肛门闭锁直肠前庭瘘1例.10例患儿均有排尿困难,其中3例伴有尿失禁.MRI显示2例合并脊髓栓系.排泄行膀胱尿道造影显示3例合并左侧输尿管Ⅳ°反流及肾积水,其中1例存在后尿道憩室,无1例发现尿道狭窄.尿动力学检查显示9例膀胱容量及残余尿增加,充盈期逼尿肌压正常,无逼尿肌过度活动,尿流率下降,其中8例逼尿肌收缩力下降,1例逼尿肌收缩力正常.另外1例直肠前庭瘘合并脊髓栓系患儿膀胱容量减少、残余尿增多、尿流率下降,充盈期逼尿肌压升高,合并逼尿肌过度活动.直肠尿道瘘合并后尿道憩室患儿行后矢状入路尿道憩室切除,泄殖腔畸形和直肠尿道前列腺部瘘术后合并输尿管反流患儿行左侧输尿管再植,8例合并神经性膀胱的患儿坚持清洁间歇导尿.结果 随访6个月～5年,泄殖腔畸形患儿1年后仍存在左侧输尿管反流及肾积水,直肠尿道球部瘘合并尿道憩室患儿输尿管反流及肾积水消失,无排尿困难及残余尿,直肠尿道前列腺部瘘合并左侧输尿管Ⅳ°反流及肾积水患儿输尿管反流消失,仍需间歇导尿,其余7例患儿无1例出现上尿路损害.结论 肛门直肠畸形合并脊髓发育不良及手术损伤可导致神经性膀胱.术中直肠尿道瘘处理不当可能导致尿道憩室或尿道狭窄.清洁间歇导尿是神经性膀胱的首要治疗方法,对于后尿道憩室可行尿道憩室切除术.     

305例原发性遗尿症儿童尿流率检测结果分析

目的通过分析尿流率检测结果评价原发性遗尿症患儿的膀胱尿道功能。方法选择2001年10月￣2005年8月在我院尿动力学室进行尿流率检测的原发性遗尿症患儿。患儿先饮水,待有强烈尿意时,在不受干扰的环境中采取自然体位排尿于尿流率测量仪器上。记录最大尿流率、平均尿流率、排尿时间、尿流时间、尿量、达峰时间及尿流曲线等,同时于肛门口贴电极片同步测量盆底肌募集肌电图。结果原发性遗尿症患儿共305例,男183例,女122例。平均年龄8.4±0.3岁(5～18岁)。白天有尿频、尿急、湿裤症状的复杂性遗尿病例225例,占73.8%;单症状性夜间遗尿病例80例,占26.2%。88.2%的患儿有效膀胱容量减小,其中单症状性夜间遗尿患儿中,82.1%存在有效膀胱容量减小,而复杂性遗尿患儿中90.0%有此现象,二者相比,差异有统计学意义(P<0.05)。7～14岁女孩最大尿流率平均为19.7±1.2ml/s,明显小于正常(P<0.05),男孩为18.6±1.1ml/s。尿流率曲线中钟形曲线占54.8%;Staccato排尿曲线占12.5%;间断排尿曲线占7.2%;功能性膀胱出口梗阻形曲线占14.4%。128例(占42.0%)患儿排尿时出现收缩的肌电图信号。结论通过尿流率分析发现部分原发性遗尿症患儿存在膀胱尿道功能异常,表现为有效膀胱容量减小、最大尿流率降低和逼尿肌-括约肌收缩不协调等。与尿动力学检查相比,尿流率检测无创易行,值得在原发性遗尿症儿童中进行。     

骶神经调节治疗青少年神经源性膀胱二例报告并文献复习

目的 探讨骶神经调节对青少年神经源性膀胱的疗效及安全性.方法 回顾性分析2013年6月至2013年11月收治的2例青少年神经源性膀胱应用骶神经调节技术治疗的临床资料,并结合文献复习讨论.2例患儿均表现为排尿费力,伴尿频及便秘,1例残余尿量120 ml,另1例残余尿量360 ml;尿动力学检查结果为逼尿肌收缩乏力.分别进行骶3神经电极植入体外测试4周,经排尿日记及尿动力学参数评估,均获得明显改善,随后行刺激器永久性植入术.结果 术后患儿排尿费力、尿频及便秘症状明显减轻,2例患儿残余尿量分别降至20 ml和50 ml.排尿日记及尿动力学参数评估(排尿量、最大尿流率、逼尿肌收缩压)较术前好转.分别随访6个月和11个月,疗效稳定,未见不良反应.结论 骶神经调节可以改善青少年神经源性膀胱的排尿及便秘症状,安全性高,但仍需大样本随机对照研究来确定此技术对儿童神经源性膀胱的长期疗效及安全性.     

后尿道瓣膜切除术后尿动力学研究

目的探讨后尿道瓣膜患儿行经尿道镜瓣膜切除术后的尿动力学改变.方法回顾性分析2007年1月至2008年12月本院收治的17例因后尿道瓣膜经尿道镜瓣膜切除术患儿的临床资料.均采取问卷调查、排尿性膀胱尿道造影、静脉肾盂造影、泌尿系超声、尿动力学检查等进行随访,重点分析尿动力学检查结果.结果诊断时常见症状排序依次为排尿困难、泌尿系感染症状、尿失禁等.术前发现肾积水17例,膀胱输尿管反流9例.均行经尿道镜瓣膜切除术.平均随访时间27(15～40)个月.临床症状均消失或减轻,无尿道狭窄、尿道瘘,造影检查提示解剖性梗阻均已解除,9例肾积水较前好转;8例存在膀胱输尿管反流.16例(94.1%)存在尿动力学异常,7例(41.2%)表现为膀胱顺应性降低,平均最大逼尿肌压力降低,逼尿肌不稳定;7例(41.2%)表现为残余尿增多.8例(47.1%)膀胱容量低于同年龄正常预测值的80%.结论后尿道瓣膜切除术后膀胱功能异常仍然存在,尿动力学检查能及时发现膀胱功能损害及其程度,建议PUV患儿术后定期行尿动力学检查,以了解膀胱功能,保护上尿路.     

尿动力学检测在儿童尿频症中的应用

目的 探讨儿童白天尿频症的尿流动力学病理改变和治疗方法.方法 随机选择40例白天尿频症患儿,进行尿动力学检测,观察尿流曲线、功能性膀胱容量(FBC)、逼尿肌稳定性及不稳定性指数、膀胱顺应性(BC)、最大膀胱测量容量(CBCmax)及CBCmax百分数、逼尿肌与尿道外括约肌协同性.在充盈性膀胱内压测定过程中当膀胱容量达正常CBCmax之前出现逼尿肌不稳定性收缩(DI)时嘱患儿收紧盆底肌、延长储尿时间,进行膀胱储尿功能训练,其中20例合并有DI的患儿在相同条件下间隔30 min再行充盈性膀胱内压测定,比较两次测定的CBCmax百分数和不稳定指数.同期选15名排尿正常儿童做对照研究.结果 膀胱充盈期出现DI 17例,DI合并低顺应性膀胱15例,单纯低顺应性膀胱4例,充盈性膀胱内压测定正常4例.3例排尿期盆底肌电活动间断增强.CBCmax下降28例.第1次CBCmax与FBC比较有明显增加(P＜0.05).20例进行2次充盈性膀胱内压测定,第2次逼尿肌不稳定指数明显下降(P＜0.05)、CBCmax百分数明显增加(P＜0.05).而正常对照组2次CBCmax无明显差异.结论 儿童白天尿频症的主要尿动力学病理变化是DI,低顺应性膀胱和CBCmax降低是DI引起逼尿肌收缩的继发改变,均为功能性紊乱,而非膀胱壁组织结构器质性病变.行为疗法是治疗儿童尿频症的有效方法,其中以排尿训练为主,抗胆碱能药可辅助治疗儿童尿频症.     

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.