小儿尿路结石的腔内微创治疗

目的探讨小儿尿路结石施行腔内微创治疗的临床价值。方法对79例小儿尿路结石患儿施行腔内微创治疗。男47例，女32例。年龄0．5～14岁，平均7．9岁。肾结石18例；输尿管上段结石14例（阴性结石和多发性结石各1例），输尿管中段结石2例，输尿管下段结石10例，膀胱内型输尿管囊肿内结石1例，肾结石合并输尿管上段结石4例；膀胱结石18例；尿道结石12例（后尿道6例，前尿道6例）。结石横径0．5～2．8cm，长径0．5～1．5cm。上尿路结石中左侧24例，右侧22例，双侧3例。结果30例下尿路结石、13例输尿管中下段结石行经输尿管镜碎石术（URL），1例肾盂结石、4例输尿管上段结石行后腹腔镜肾盂输尿管切开取石术（RPPL／RPUL），31例肾、输尿管上段结石行微创经皮肾镜取石术（MPCNL），手术均获成功。术中、术后无严重不良反应发生。结论综合应用各种腔内治疗技术，小儿尿路结石可以实现现代微创治疗，并可作为一线治疗的选择。     

经输尿管镜钬激光碎石术治疗婴幼儿输尿管结石

目的 探讨经输尿管镜钬激光碎石术治疗婴幼儿输尿管结石的可行性和疗效.方法 回顾性分析2008年12月至2010年10月36例41侧婴幼儿输尿管结石采用经输尿管镜钬激光碎石术治疗的临床资料.结果 36例41侧输尿管结石,其中32侧一次入镜碎石成功;8侧采用导丝、输尿管导管、双J管扩张后碎石成功;1侧改经皮肾穿刺钬激光碎石术.所有病例术后均留置双J管1～4周,拔管后2周内复查B超或KUB结石全部排尽,清石率97.6％.7侧合并有输尿管息肉者,息肉切除.发生输尿管口撕裂1例,黏膜下假道2例,留置双J管4周,无输尿管反流和狭窄并发症.1例出现术后发热,经抗感染后体温正常.所有病例术后随访3～24个月未见输尿管狭窄和结石复发.结论 选择合适的输尿管镜,熟炼掌握操作技术,采用经输尿管肾镜钬激光碎石术治疗婴幼儿输尿管结石安全有效.     

输尿管镜术治疗学龄前儿童输尿管中下段结石

目的 评价输尿管镜取石术治疗学龄前儿童输尿管中下段结石的疗效与安全性。方法 2001年2月-2006年4月，13例学龄前儿童输尿管中下段结石患儿接受不同方式输尿管镜术治疗，回顾性分析其临床资料。结果 13例患儿中，1例输尿管末端结石患儿逆行入镜及置入导丝失败改开放手术，2例行Ⅰ期6．5／8．5Fr输尿管半硬镜下钬激光碎石，2例行Ⅰ期硬性扩张后8／9．8Fr输尿管硬镜取石，1例行Ⅰ期气囊扩张后8／9．8Fr输尿管硬镜下取石，7例Ⅰ期输尿管置管被动扩张1～3周后，Ⅱ期8／9．8Fr输尿管硬镜下取石。12例息儿共接受19次输尿管镜术，结石全部取出，无输尿管穿孔等严重并发症。随访3～12个月，4例（1例I期硬性扩张，3例置管被动扩张）患儿接受排尿性膀胱造影未见膀胱输尿管反流，5例（2例Ⅰ期6．5／8．5Fr输尿管半硬镜下钬激光碎石术，3例Ⅰ期置管被动扩张）行静脉肾盂造影未见输尿管开口狭窄及肾积水。结论 输尿管镜术治疗学龄前儿童输尿管中下段结石直接明了，安全有效，作为ESWL的有益补充。     

无管化经尿道输尿管镜下2μm激光联合第四代EMS系统治疗小儿输尿管囊肿合并结石

目的探讨经尿道输尿管镜下2μm激光联合第四代EMS治疗小儿输尿管囊肿合并结石的可行性。方法对23例接受经尿道输尿管镜下2μm激光低位开窗+碎石清石术治疗的小儿输尿管囊肿合并结石患儿的临床资料进行回顾分析。患儿年龄1～8岁,中位年龄2.5岁。经尿道置入F8/9.8输尿管镜,以2μm激光手术系统行囊肿低位开窗术,再以EMS系统碎石清石。术后不留置输尿管支架管及尿管,观察临床效果。结果全部病例顺利完成手术,术后无明显并发症,如期出院。随访期间未见输尿管囊肿或结石复发。结论经尿道输尿管镜下2μm激光联合第四代EMS系统治疗小儿输尿管囊肿合并结石具有出血少、操作安全简便等优点,无管化方案可进一步减少手术创伤,减轻术后不适感,加快康复进程,是一种安全、可靠的微创手术方法。     

输尿管镜气压弹道碎石术治疗小儿输尿管结石

目的探讨输尿管镜气压弹道碎石术治疗小儿输尿管结石的临床效果。方法回顾性分析应用输尿管镜气压弹道碎石术治疗小儿输尿管结石32例临床资料。结果一次性治疗成功29例，成功率90．6％，手术时间为30～120min，术后无明显血尿、感染及输尿管、尿道狭窄等并发症。结论输尿管镜气压弹道碎石术治疗小儿输尿管结石效果确切、创伤小，是治疗小儿输尿管结石的理想方法。     

输尿管镜气压弹道碎石术治疗小儿下尿路结石疗效观察

目的 探讨经输尿管镜气压弹道碎石术在治疗小儿下尿路结石中的应用效果.方法 收集我科2012年1月至2015年1月33例小儿下尿路结石患儿,其中男32例,女1例,年龄6个月至12岁,平均年龄29个月,年龄小于3岁的患儿有27例.膀胱结石17例,尿道结石16例,其中前尿道结石6例,后尿道结石10例,结石大小8 mm～25 mm,平均13 mm.所有患儿经B型超声、泌尿系CT等检查,均无尿道狭窄、神经源性膀胱、先天性畸形.全身麻醉、电视监视系统下应用Wolf输尿管镜(Wolf 6～7.5F,工作通道F4.0)气压弹道碎石术(碎石杆直径1.0 mm)治疗,观察碎石成功率、术后有无尿道损伤、膀胱穿孔、有无发热等并发症发生情况及结石复发情况.结果 33例患儿均1次碎石成功,碎石时间15～60 min,平均(26±9)min,术后2d～7d内结石清除率100％,4例患儿术后第一天体温超过38.0℃、无“石街”、无尿道狭窄、无尿道损伤、无膀胱穿孔和排尿异常等并发症发生,术后住院时间2～3 d,平均2.4d.随访2～24个月,无结石复发.结论 经输尿管镜气压弹道碎石术治疗小儿尿路结石安全简便、效果确切、结石清除率高,且损伤小,术后恢复快,可作为小儿下尿路结石治疗的首选方法之一.     

经输尿管软镜钬激光碎石术治疗儿童肾结石和输尿管上段结石

目的 探讨经输尿管软镜钬激光技术治疗儿童肾结石和输尿管上段结石的疗效.方法 回顾性分析47例（52侧）采用经输尿管软镜钬激光技术治疗的上尿路结石患儿临床资料.结果 47例52侧上尿路结石,单用输尿管软镜碎石取石术35例、硬镜＋软镜12例.其中2侧一次入鞘置镜碎石成功;其余采用双J管扩张输尿管后,45侧置入软镜导引鞘成功.45侧置入导引鞘者,39侧一次碎石成功;另外3侧进行了第二次碎石手术;其他3侧未寻及结石,2侧改由MPCNL,1侧观察.5侧导引鞘置入不成功者,其中2侧在斑马导丝引导下置入软镜到肾盂进行碎石成功;另外2例改由微创经皮肾穿刺碎石取石术（MPCNL）,1例失访.共49侧入镜成功并碎石.术后1个月复查B超或CT,46侧结石全部排尽,清石率88.5%（46/52）,3例残留下盏结石,术后2～4周再行软镜碎石取石术,术后1个月复查,结石全部排尽.术后2个月总清石率92.3%（49/52）.本组平均手术时间为 45 min（25~115 min）.术后平均住院时间3 d（2~5 d）,术后出血少,发生输尿管口撕裂1例,无输尿管反流和狭窄.3例出现术后发热.43例术后随访2～24个月,未见输尿管狭窄,1例术后1年结石复发.结论 选择合适的输尿管软镜,熟炼掌握操作技术,采用经输尿管软镜钬激光碎石术,是治疗儿童肾和输尿管上段结石安全有效的方法.     

输尿管镜钬激光治疗儿童输尿管末端囊肿的疗效分析

目的：探讨输尿管镜钬激光治疗儿童输尿管末端囊肿的疗效。方法回顾性分析2010年3月至2014年3月本院22例儿童输尿管末端囊肿临床资料,均经B 超、泌尿系CT、静脉尿路造影、膀胱逆行造影检查确诊,并接受输尿管镜钬激光囊肿切开术。22例患儿年龄3个月至15岁,平均3.6岁,其中女性15例（68.2％）,男性7例（31.8％）;左侧9例（40.9％）,右侧11例（50.0％）,双侧2例（9.1％）;囊肿开口于膀胱内20例（90.9％）,开口于膀胱颈尿道内口2例（9.1％）;16例（72.7％）合并患侧肾积水或者输尿管扩张,7例（31.8％）合并重复肾畸形,且均为上下位肾输尿管Y形融合并以末端囊肿开口于膀胱内,4例（18.2％）伴有膀胱输尿管反流,1例（4.5％）合并输尿管末端结石,13例（59.1％）合并尿路感染;均行输尿管镜钬激光囊肿切开术,手术时间为14～46 min,平均（28±10）min,术中出血量2～10 mL,住院时间2～5 d,平均（3.1±1.0）d。结果术后21例随访,1例失访,随访时间为3～31个月,20例未见输尿管囊肿复发,10例尿路感染症状完全消失,12例肾积水或者输尿管扩张得到明显改善,1例合并结石的未见复发,1例出现膀胱输尿管反流加重而行输尿管膀胱再植术。结论输尿管镜钬激光治疗儿童输尿管末端囊肿操作简便,创伤小,手术时间短,恢复快,并发症少,可作为首选治疗方式;对于严重膀胱输尿管反流,异位输尿管开口的重复肾患儿,术前需要做充分评估,以减少再次手术的风险。     

钬激光在治疗小儿尿道狭窄中的应用

目的探讨钬激光在治疗小儿尿道狭窄中的应用效果。方法我们对21例尿道狭窄．2例后尿道瓣膜患儿经膀胱镜用钬激光行狭窄切开及瘢痕或瓣膜切除,并随访观察疗效。结果22例患儿一次治愈,仅1例因狭窄段过长复发,经再次钬激光切除治愈。结论钬激光是一种安全、高效、微创的治疗儿童尿道狭窄的新型手术方法。     

小儿输尿管结石的输尿管镜和ESWL治疗

目的 报告小儿输尿管结石URS和ESWL治疗经验，探讨微创治疗的效果和策略。方法 复习了1988～2003年间65例小儿输尿管结石的治疗和结果，其中22例患儿应用ESWL治疗；43例输昧管中、下段结石和5例上段嵌顿结石FSWL失败者进行了输尿管镜治疗，用APL弹道碎石机或钬激光击碎结石并取出，7例联合ESWL；1例接受了开放手术．结果 22例输尿管上段结石经过29次ESWL治疗，随访3个月，结石完全排空16例，完全排空率为72．7%。43例输尿管中、下段结石和5例上段嵌顿结石ESWI．失败者进行了48次输尿管镜治疗，47次进镜治疗成功。30例下段结石URS治疗均成功；13例中段结石，10例URS取石成功。3例中段结石和4例上段结石联合ESWL碎石均成功，45例术后停留引流管1～4周，随访3个月，结石清除率为979％。1例上段结石入镜失败改行开放手术取石。结论 ESWL仍是输尿管上段结石的首选治疗方法；对输尿管中、下段结石特别是下段结石应用小直径输尿管镜则是安全有效的方法；部分中、上段结石采用URS联合ESWL效果可能更好。     

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   

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.     

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.     

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.     

Psychopathologie du syndrome d’Asperger et « aspérigisation » de la société contemporaine

The author has attempted here to point out, just for a start, the characteristics of Asperger syndrome from the point of view of psychopathology through a rereading of Hans Asperger's original paper (1944). This thesis merits reevaluation, if for no other reason than to fill the gaps in operational diagnostics based on the DSM. It is found by rereading that Asperger's view of the principal disturbances of autistic psychopathy include a “disturbance of natural evidence” or a “crisis of common sense”. This question of natural evidence that he evokes with regard to autistic psychopathy corresponds to W. Blankenburg's natural evidence, which constitutes a key concept for comprehending schizophrenia in the form poor-symptom (“symptomarme Schizophrenie”) that he observes in the speech of his patient Anne Rau. One can deduce from this that in terms of fundamental disturbances, Asperger syndrome and this “symptom-poor” schizophrenia overlap at the level of loss of natural evidence. It is moreover possible to classify Asperger syndrome among the disturbances of spacing in the sense meant by the evolutionary psychiatry of A. Stevens and J. Price. The author then develops our comprehension of Asperger syndrome from the point of view of the perspective proposed by the notion of resilience in people with Asperger syndrome and of the possibility for them, through these mechanisms of adaptation, to find in the organization of the personality of the “as if” type a position of relative equilibrium. They concur or overlap in the creation of crutches, of borrowed personalities secondarily legitimated by the reaction of the socius. This will end up in the production of inventions and œuvres (works). Clearly, one rarely encounters several cases that one could consider pertinently to be “successful” Asperger syndrome. Finally, the author notes that one can find a sort of isomorphism between Asperger syndrome and contemporary society when he proposes the term “asperigisation” to characterize our society, given that the equilibrium between emotion and logic is strongly disturbed in these patients, in whom logic undergoes hypertrophy while emotion is impoverished. From this perspective, the author hopes to suggest reasons for the increase in the number of cases of Asperger syndrome in the clinical setting and in society in general in our contemporary era.     

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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     

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.     

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.