隐睾合并附睾畸形与雌激素的关系

探讨隐睾儿童血清雌激素与隐睾、附睾畸形三者之间的关系。我们对６４例隐睾儿童手术前采血，用放射免疫分析法测定血清雌二醇（Ｅ２）值，与６８例健康男孩对照比较（ｔ检验）。术中观察睾丸和附睾之间的附着形态。结果：①隐睾儿童血清Ｅ２值（１４３．９０±１２４．１５ｐｍｏｌ／Ｌ）明显高于正常对照组（４５．４８±３９．１３ｐｍｏｌ／Ｌ），Ｐ＜０．０１；②隐睾合并附睾和（或）输精管畸形者占３９．１％，且隐睾合并附睾畸形组与不合并附睾畸形组比较，血清Ｅ２值明显增高；③高位和腹股沟管型隐睾分别与皮下环型比较，血清Ｅ２明显增高，因此，隐睾胚胎期Ｗｏｆｆｉａｎ管的分化发育与血清Ｅ２水平之间存在某种内在性联系，可能是隐睾的重要发病因素。     

青春期前隐睾患儿手术前后抗精子抗体检测的意义

目的检测青春期前隐睾患儿手术前后血清抗精子抗体（AsAb），探讨AsAb与睾丸位置、手术、单／双侧和伴输精管／附睾畸形等因素的关系。方法收集隐睾患儿,50例，取腹股沟斜疝患儿和腹股沟区正常儿童各50例作为对照组。分别抽取患儿术前及术后6个月血清，采用AsAb金标免疫斑点法检测其血清AsAb IgG、IgM水平。结果隐睾组术前及行睾丸下降固定术后AsAb的阳性率分别明显高于腹股沟斜疝组术前及术后和健康对照组（Pa〈0．01）。睾丸下降固定术、单／双侧隐睾、术前睾丸位置及伴输精管和（或）附睾畸形均与AsAb无显著相关（Pa〉0．05）。结论青春期前隐睾患儿血清AsAb阳性率显著升高，与手术、术前睾丸位置、单／双侧隐睾及是否伴输精管／附睾畸形无显著关系。     

人绒毛膜促性腺激素对单侧隐睾大鼠睾丸生殖细胞凋亡的影响

目的探讨人绒毛膜促性腺激素（HCG）对单侧隐睾大鼠睾丸生殖细胞凋亡的影响。方法将sD雄性大鼠40只随机分为单侧隐睾组和假手术组，各20只，于日龄21d制备单侧隐睾模型。单侧隐睾组和假手术组各又分为HCG治疗组和未治疗组。日龄22d时HCG治疗组开始肌肉注射HCG20U，隔日1次，共7次。日龄35、60d时处死大鼠，取其睾丸，采用生物素-dUTP／酶标亲和素法（TUNEL法）检测其生殖细胞凋亡水平。结果单侧隐睾组隐睾睾丸生殖细胞凋亡指数（AI）高于假手术组，但无统计学差异（P〈0．05）；单侧隐睾各组对侧睾丸生殖细胞AI高于假手术未治疗组（P〉0．05）。假手术和单侧隐睾模型HCG治疗组阴囊内睾丸生殖细胞AI高于相应未治疗组，且35d假手术组治疗组与未治疗组间差异有统计学意义（P〈0．05）。60d单侧隐睾HCG治疗组大鼠隐睾侧睾丸生殖细胞AI高于相应未治疗组，但无统计学差异（P〉0．05）。结论单侧隐睾时不仅患侧睾丸生殖细胞凋亡增加，而且隐睾对侧阴囊内睾丸生殖细胞凋亡也增加；HCG的应用可加重睾丸生殖细胞凋亡，且停用后仍存在着一些不可逆的病理损害，故临床应用HCG要慎再，应尽早手术治疗。     

胰岛素样因子3与隐睾发病的相关性研究进展

隐睾（Cryptorchidism）是小儿时期最常见的泌尿生殖畸形，在足月男婴中的发生率高达4％，它不仅是引起男性生殖功能障碍、产生不育的主要原因，而且隐睾的恶变率也较正常睾丸高出20～46倍。     

微创经皮肾取石治疗小儿复杂肾结石

目的总结小儿复杂肾结石微创治疗经验，探讨小儿肾结石微创经皮肾取石（mini—PCNL）的治疗效果。方法回顾了10年间（1995-2005年）行mini—PCNL的35例患儿的临床资料，男20例，女15例，平均年龄8岁（3．5～14岁），其中伴尿路畸形8例（22．9%），3例伴UPJ梗阻同时行顺行肾盂输尿管狭窄内切开术，1例UPJ梗阻输尿管镜扩张，4例联合ESWL。结果Ⅰ期mini-PCNL为26例（74．3％），Ⅱ期mini—PCNL为9例（25．7％）；Ⅰ期结石完全清除25例（71．4%），Ⅱ期结石完全清除6例（17．1％），联合ESWL4例（11．4％），总的结石清除率为97．1％。手术时间平均40min（30-120min）；平均住院时间7d（4～15d）。4例UPJ梗阻治疗效果良好。结论微创经皮肾取石治疗小儿复杂肾结石是安全有效的方法，但要根据结石和设备技术情况，联合ESWL成功率更高。     

肾母细胞瘤血清蛋白质标记物检测与分期模型构建研究

目的筛选出肾母细胞瘤患儿特异性血清蛋白质标记物，建立肾母细胞瘤临床分期模型与CT分期进行对照分析，并评价其临床应用价值。方法应用SELDI-TOF-MS技术检测80例血清标本（术前肾母细胞瘤30例，其他恶性肿瘤30例，正常儿童20例），用ZUCI-Protein Chip Data Analyze System分析软件进行数据处理，结合支持向量机（support vector machine，SVM）建立肾母细胞瘤临床分期模型。结果筛选出2个m／z位于4153．9和3257．6的蛋白质标记物，区分肾母细胞瘤Ⅲ和Ⅳ期与肾母细胞瘤Ⅰ和Ⅱ期蛋白质谱差异表达模型的敏感性为100％，特异性为93。8％；区分肾母细胞瘤与正常儿童、腹腔实体肿瘤及肾脏其他恶性肿瘤的特异性是100％，敏感性是100％、80．0％、100％；临床分期模型可以特异性地将各期区分开来，其特异性及敏感性均为100％；通过肾母细胞瘤早期诊断模型中的2个m／z（6984．4，6455．5）血清标记物进行分析得出肾母细胞瘤各期情况如下：Ⅳ期相对于Ⅲ期低表达；Ⅲ期相对于Ⅱ期低表达；Ⅱ期相对于Ⅰ期低表达；Ⅰ期相对于正常儿童低表达；后者相对高表达；临床分期越晚，m／z强度就越低表达。蛋白芯片分期准确性与病理一致，达到100％，在分期定性问题上优于CT（Ⅰ期100％，Ⅱ期85．0％，Ⅲ期85．0％，Ⅳ期75．0％）。结论用SELDI-TOF-MS结合SVM建立的肾母细胞瘤临床分期模型可弥补CT在肾母细胞瘤分期定性问题匕的不足。     

儿童感染性心内膜炎临床特点变化及病原学变迁

目的分析儿童感染性心内膜炎临床特点变化及病原菌的变迁，为临床诊治提供进一步指导。方法回顾分析10年中我院73例14岁以下儿童感染性心内膜炎患儿外科手术的临床资料。并将1995年1月-1999年12月的32例（Ⅰ组）与2000年1月-2004年12月的41例（Ⅱ组）做统计学分析对比，比较两组病例临床特点的变化及病原菌的变迁。结果73例患儿占同期同年龄组住院患儿总数的7．3％（73／998），Ⅱ组的比例（41／671，6．1％）低于Ⅰ组（32／327，9．8％，P〈0．05）。66例有基础心脏病，其中室间隔缺损（VSD）47例，动脉导管未闭（PDA）15例，其他4例。心脏赘生物的检出率，Ⅱ组的比例（27／41，65．9％）高于Ⅰ组（15／32，46．9％，P〈0．01）。59例血细菌培养阳性，14例血细菌培养阴性。总的细菌培养阳性率为73．9％（54／73），Ⅰ组为96．88％（31／32），Ⅱ组为68．29％（28／41）。心脏赘生物的检出率增高，由Ⅰ组的46．9％（15／32）增高为Ⅱ组为65．9％（27／41）。儿童IE的病原菌菌谱发生明显变化，革兰阳性球菌的阳性检出率明显减少，而革兰阴性杆菌的检出率明显增加。结论外科治疗的儿童感染性心内膜炎临床特点发生一定变化，血培养的阳性率降低，而心脏赘生物的检出率增高。儿童IE的病原菌菌谱中革兰阴性杆菌所占比例有明显的增高。     

小儿未触及隐睾的诊断和治疗

目的探讨小儿未触及隐睾的定位诊断和治疗方法。方法对64例75侧未触及隐睾的14岁以下手术病例作回顾性分析。结果B超探查未触及隐睾的假阴性率为82．8％(48／58)。行肉膜囊睾丸固定术52侧，Fowler-Stephens手术8侧，睾丸切除术4侧，分期睾丸固定术2侧，未找到睾丸9侧。需要广泛手术探查的腹内高位隐睾或无睾仅8侧。术后随访88．5％的睾丸位置良好，无进一步萎缩。结论手术是未触及隐睾定位和治疗的主要方法。探查首选经腹股沟入路，经正确的术中处理大多数小儿未触及隐睾可经Ⅰ期手术降入阴囊。     

腹腔镜诊治高位隐睾51例

目的探讨腹腔镜技术诊断和治疗高位隐睾的临床疗效。方法2005年7月至2008年7月，作者对51例（69侧）高位隐睾利用腹腔镜技术进行诊断和治疗。结果51例均得到明确诊断，69侧中，13侧为睾丸缺如。41侧行一期睾丸固定术，15侧行分期Fowler-Stephens睾丸固定。49例获随访0．5～3年，睾丸无回缩或萎缩，2例失访。结论腹腔镜诊断和治疗高位隐睾可行、有效。     

小儿肾母细胞瘤146例分析

目的总结小儿肾母细胞瘤的治疗经验,以期改进肾母细胞瘤的治疗方法,改善。肾母细胞瘤患儿的预后。方法回顾性分析本院近20年来收治的146例。肾母细胞瘤患儿的临床资料。结果146例中,Ⅰ期62例,Ⅱ期32例,Ⅲ期33例,Ⅳ期17例,Ⅴ期2例：71例随访时间〉5年,共死亡11例,无瘤存活60例,总治愈率84．5％（60／71）,其中Ⅰ期97．3％（37／38）,Ⅱ期83．3％（10,12）,Ⅲ期72．7％（8／11）,Ⅳ期55．5％（5／9）。对术前评估手术切除困难或有远处转移的48例进行术前化疗5周,其中41例一般情况明显改善,肿瘤缩小;4例肿瘤体积增大;3例下腔静脉瘤栓患儿死亡;45例行手术完整切除肿块。结论采取综合治疗后小儿肾母细胞瘤的治愈率得到明显提高,其中手术加规范的化疗起关键作用。     

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.