先天性尿道下裂术后尿瘘防治的探讨

目的探讨减少尿道下裂术后尿瘘发生的措施及尿瘘修补方式。方法我们对200例近端型尿道下裂患儿，行Duckett改良术；对术后尿瘘患儿采用原位皮瓣加临近皮下组织瓣覆盖术进行修补。结果改良Duckett术后尿瘘16例，采用加盖方式进行尿瘘修补术一次成功率100％。结论改良Duckett能取得良好的疗效，可减少尿瘘发生率，是减少尿瘘发生较好的方式；采用原位皮瓣加临近皮下组织瓣覆盖提高了尿瘘修补的成功率。     

尿道下裂术后尿瘘的修补

尿道下裂修复术后尿瘘是最常见的并发症 ,手术修补失败率在 40 .0 %左右[1] 。我院近 2 0年收治尿道下裂修复术后尿瘘 10 2例 ,一次修补成功率82 .3%。本文就手术后修补方式和失败原因进行探讨 ,现报告如下。资料方法一、一般资料10 2例中 ,年龄 1～ 3岁 19例 ,4～7岁 5 6例 ,8～ 10岁 9例 ,11～ 14岁18例。尿道下裂部位 :阴囊型 19例 ,阴茎阴囊型 5 1例 ,阴茎型 14例 ,冠状沟下18例。尿瘘发生部位及首次手术修复方式 :冠状沟下 32例 ,用带蒂包皮瓣修复 2 5例 ,阴囊中膈瓣修复 7例。阴茎中部 2 0例 ,阴茎中部皮瓣修复 17例 ,带蒂包皮瓣修复 …     

445例先天性尿道下裂的治疗

目的 探讨不同术式对445例不同类型先天性尿道下裂患儿的手术治疗效果。方法 回顾性分析我院1988年—2003年445例先天性尿道下裂病例资料，其中采用阴茎阴囊纵隔带蒂皮瓣尿道成型术311例，包皮内板带蒂皮瓣尿道成型术52例，包皮及阴茎阴囊联合皮瓣尿道成型术61例，膀胱粘膜代尿道术21例。每例均同时行耻骨上膀胱造瘘术。结果 治愈率90．6％，并发症为9．4％。其中尿道狭窄11例，尿瘘31例。尿道狭窄中膀胱粘膜法1例，包皮内板法2例，阴茎阴囊纵隔法5例，联合皮瓣法3例；尿瘘中膀胱粘膜法1例，包皮内板法3例，阴茎阴囊纵隔法24例，联合皮瓣法3例。结论 根据尿道下裂类型选择不同的术式 尿流改道，并严格遵守整形外科的原则是尿道下裂手术成功的关键。     

筋膜瓣技术在尿道下裂手术中的应用

目的探讨筋膜瓣技术在尿道下裂术中应用于创面修复的方法及可行性。方法收集先天性尿道下裂中需行尿道重建的患儿603例,年龄2～18岁。486例为首次手术病例,117例为再次手术病例。按尿道下裂病变特点分别采用Mathieu、带蒂岛状皮瓣法、Snodgrass、Duckett、Duckett+Duplay、Duplay、Thiersch术及膀胱黏膜法等重建尿道。按治疗年限及尿道创面覆盖方法分为筋膜瓣组360例(分别应用尿道自身筋膜瓣、阴茎旋转筋膜瓣、阴茎去上皮筋膜瓣、阴囊轴形筋膜瓣及任意筋膜瓣覆盖新建尿道,再行皮瓣转移错位缝合覆盖阴茎创面)和皮瓣组243例(直接应用皮瓣转移覆盖阴茎创面)。患儿均采用相同缝线及尿道支架材料。术后抗炎,10～14 d拔管。结果各种尿道重建术式中筋膜瓣组尿瘘的发生率均低于皮瓣组。除Duplay及膀胱黏膜法两术式外,其余术式2组尿瘘率比较差异均有统计学意义(Pa<0.05)。筋膜瓣组37例发生尿瘘,无尿道全程裂开等严重并发症病例,总尿瘘率为10.3%;皮瓣组77例发生尿瘘,其中17例新建尿道全程裂开,总尿瘘率为31.7%,2组总尿瘘率比较差异有统计学意义(P=0.000)。结论筋膜瓣技术适用性广、取材灵活,可为尿道提供良好的组织覆盖,有效避免尿瘘的发生,可作为尿道下裂术中创面修复的常规方法。     

改良Snodrass手术一期修复小儿尿道下裂25例

目的总结改良Snodgrass术式在小儿尿道下裂治疗中的经验。方法对25例尿道下裂患儿（含阴茎体型15例，冠状沟型10例）采用改良Snodgrass术式行一期修复手术，评价疗效，并分析其手术方法。结果术后随访2个月至4年。21例一期手术治愈；3例出现尿瘘（12％），经尿瘘修补手术后治愈；2例卅现尿道狭窄，经尿道扩张治疗后痊愈（8％）。结论改良Snodgrass手术操作简单，适用于阴茎体型和冠状沟型尿道下裂。     

应用血管蒂筋膜覆盖--预防尿道下裂术后尿瘘的一种有效措施

目的 分析在应用带血管蒂的皮瓣尿道成形术中，应用血管蒂筋膜覆盖尿道的要点，探讨提高治愈率的措施。方法 改进包皮岛状皮瓣及阴囊中隔带蒂皮瓣尿道成形术。在完成常规步骤后，将血管蒂筋膜缝合固定在阴茎白膜及尿道海绵体上，使尿道完全被血管蒂筋膜覆盖，增加了覆盖在尿道表面组织的厚度及血供。对是否使用血管蒂筋膜覆盖病例的Ⅰ期治愈率等效果进行统计学分析。结果 与对照组相比，应用血管蒂筋膜覆盖者，包皮岛状皮瓣尿道成形术Ⅰ期治愈率由64．8％提高到93．3％（P〈0．01），尿道瘘发生率南24．1％降到1．9％。阴囊中隔皮瓣尿道成形术Ⅰ期治愈率由84．2％提高N95．7％（P〈0．01），尿道瘘发生率由7．0％降到1．2％。结论 应用血管蒂筋膜覆盖尿道能增加新形成尿道及吻合口的组织覆盖面及厚度，保证良好的血供，是减少尿道下裂术后尿道瘘的一种有效措施。     

阴囊中缝带蒂皮瓣转移覆盖在尿道下裂多次手术皮肤缺损患儿中的应用

目的分析重度尿道下裂多次手术皮肤缺损患儿采用口腔黏膜镶嵌式尿道成形阴囊中缝带蒂皮瓣转移覆盖术的治疗效果。方法收集2013年10月至2016年10月间入住本院的16例尿道下裂手术治疗失败需再次手术病例,年龄4~13岁,平均年龄7.5岁。尿道重塑均采用口腔黏膜镶嵌尿道成形术,再游离阴囊中缝带蒂皮瓣将之转移至阴茎腹侧创区皮肤缺损处,均顺利完成手术。结果16例患儿术后均通过电话预约门诊复查完成随访,随访时间12~24个月,平均随访时间为15个月。患儿阴囊中缝转移皮瓣均成活,排尿顺畅,尿道外口位置良好,无尿道憩室和尿道瘘发生,阴茎阴囊外观患儿家长满意。结论多次进行手术修补失败的尿道下裂患儿采用口腔黏膜镶嵌尿道成形术重塑尿道后联合阴囊中缝带蒂皮瓣转移覆盖术治疗,术后患儿阴茎阴囊外观满意,无尿道憩室尿道瘘发生,疗效满意,治愈率高,值得临床推广应用。     

带蒂包皮双面皮瓣法联合DuplayⅠ期修复重度尿道下裂

目的 总结带蒂包皮双面皮瓣法(PPDIF)联合原位尿道板卷管(Duplay)治疗重度尿道下裂的方法及疗效.方法 1998年1月至2010年3月采用带蒂包皮双面皮瓣法加原位近端尿道板卷管(PPDIF+ Duplay)术式治疗重度尿道下裂72例,包括阴囊型50例,会阴型22例.结果 平均随访23个月,术后阴茎伸直及外观满意,尿道口位置正常.发生尿瘘10例(13.9％),尿道狭窄4例(5.6％),手术总成功率达80.6％.结论 PPDIF联合Duplay术式 Ⅰ期修复重度尿道下裂疗效肯定,具有术后外形美观,尿道狭窄、尿瘘等并发症少等优点.     

尿道下裂术后尿瘘原因及修补体会

目的 探讨各型尿道下裂术后尿瘘发生原因及修补方法。方法 小尿瘘采用结扎法，切开缝合法，皮瓣覆盖法修复，大尿瘘采用Thiersch法或重新行尿道成形术。结果 123例尿道下裂术后29例出现尿瘘(23．58％)。尿瘘发生后立即修复的成功率(3／9，33．33％)远低于尿瘘发生半年后修复的成功率(14／17，82．35％)。3例半年内自行愈合。结论 尿瘘的发生与尿道成形材料，局部血运及创面感染有关，与术式本身关系不大。瘘口大小、术式选择、修补时间是尿瘘修补成功的关键。     

尿道下裂尿道成形术后尿瘘的处理

目的探讨尿道下裂尿道成形术后尿瘘的处理。方法总结1994-2003年464例尿道下裂尿道成形术后尿瘘治疗的临床经验。年龄1～18岁，平均6．5岁。2/3患儿来自外院。大尿瘘163例使用Thierseh、Snodgrass、Duckett、Duplay、Mathieu、Onlay、Denis—Browne和阴囊中隔修补术。小尿瘘301例用简单切开缝合法和Y-V皮瓣覆盖尿瘘修补法。结果术后随访6个月～10年，一次修补成功率93．3％，其巾大瘘85．9％，小瘘97．3％。结论尿道下裂Ⅰ期手术方法的正确选择对提高尿瘘修补手术成功率至关重要。应该按照尿瘘的大小、位置、数量和局部条件选择不同修瘘手术方法。     

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.