小儿完全性肺静脉畸形引流的外科治疗

目的　评判完全性肺静脉畸形引流早期手术矫治的疗效及心上型完全性肺静脉畸形引流各种吻合方法的优缺点。方法　 1976～ 1999年共收治完全性肺静脉畸形引流 14 0例 (男 81,女5 9) ,平均年龄 ( 7.13± 6.88)岁。心上型 87例 ( 62 .14 % ) ,心内型 4 4例 ( 3 1.4 3 % ) ,心下型 2例( 1.4 3 % ) ,混合型 7例 ( 5 % )。 2例经右心导管检查证实为重度肺动脉高压 ,未能实施矫治手术 ,余患儿均在全麻、低温、体外循环下行矫治术。心上型的吻合方法包括 :心外法 ( 2 1例 ) ,经上腔静脉与升主动脉间吻合法 ( 3例 ) ,经房间隔缺损吻合法 ( 18例 ) ,心外吻合加经房缺吻合 ( 2例 ) ,经左右心房联合切口吻合法 ( 4 3例 )。结果　术后早期 ( 3 0d)死亡 14例 ( 10 % ) ,其中 3例合并其他复杂的心内畸形 ,均死亡 ,故单纯TAPVC的手术死亡 11例 ,死亡率为 8.0 3 % ( 11/ 13 7)。术后主要并发症为心律失常 60例 ( 4 2 .86% )。结论　完全性肺静脉畸形引流宜尽早手术 ,心上型矫治术吻合口足够大是保证手术成功的关键 ,左右房联合切口入路可确保吻合口足够大 ,但心律失常发生率高 ,经上腔静脉与升主动脉间吻合法可减少心房损伤 ,降低心律失常发生率。     

体重5kg以下完全性肺静脉异位引流患儿的外科治疗

目的 探讨体重5 kg以下完全性肺静脉异位引流患儿的外科治疗效果.方法 2002年1月至2009年12月,首都医科大学附属北京儿童医院外科手术矫治体重5 kg以下各型完全性肺静脉异位引流患儿52例,均在全麻、低温体外循环下手术.对心上型采取经左、右心房联合切口或心上入路完成肺静脉共腔与左房的侧侧吻合;心内型剪除冠状静脉...     

采用左心房顶部进路纠治心上型完全性肺静脉异位连接

目的　评估经左房顶部进路方法纠治心上型完全性肺静脉异位连接。方法　 2 0 0 1年11月至 2 0 0 3年 1月 ,采用经左房顶部进路方法纠治心上型完全性肺静脉异位连接 16例。年龄 (2 3d～ 4岁 ) ,其中 6个月以下 8例 ,占 5 0 .0 % ;平均体重 (8.5± 4 .94 )kg ,10kg以下共 11例 ,占全组6 8.7%。结果　纠治心上型完全性肺静脉异位连接 16例 ,无死亡。其中 1例术后吻合口狭窄 ,第 2d再次手术 ,肺静脉回流通畅 ,康复出院。 1例婴儿术后做膈肌折叠术。 2例新生儿术后延迟关胸。术后随访 3～ 16个月 ,所有患儿生长发育良好 ,胸片示心影较术前明显缩小 ,肺充血消失 ,心电图示窦性节律 ,无一例出现肺静脉回流梗阻。结论　采用经左房顶部进路方法纠治心上型完全性肺静脉异位连接 ,取得较好效果。手术成功取决于左房与汇总静脉的吻合口大小 ,保证肺静脉回流无梗阻。     

心上径路治疗新生儿和小婴儿完全性心上型肺静脉异位引流

目的 探讨心上径路治疗新生儿和小婴儿完全性心上型肺静脉异位引流(total anomalous pulmonary venous connection,TAPVC)的临床效果.方法 2006年6月至2009年6月,采用心上径路矫治新生儿和小婴儿心上型TAPVC 26例,其中男17例,女9例.年龄18 d～8个月,平均(4.2±2.8)个月.体重3.4～8.1 kg,平均(5.8±2.1)kg.全部患儿经心上径路完成矫治手术.总转流时间49～98 min,平均(68.7±15.3)min;主动脉阻断时间31～77 min,平均(47.6±14.7)min.结果 术后早期无死亡;交界区心律或心律紊乱5例(19.1%);中期随访23例,时间5个月～3年,平均18个月,无死亡.心功能Ⅰ级21例,Ⅱ级2例,全部为窦性心律;复查超声心动图示吻合口通畅22例,轻度狭窄1例.结论 采用心上径路治疗新生儿和小婴儿完全性心上型肺静脉异位引流临床效果满意.

Abstract：

Objective To evaluate the short term outcome of surgical treatment of supracardiac total anomalous pulmonary venous connection (TAPVC) via superior approach in neonates and infants. Methods From June 2006 to June 2009, 26 patients with supracardiac TAPVC underwent surgical repair through superior approach. There were 17 males and 9 females. Their ages ranged from 18 days to 8 months old (mean, 4. 2 ± 2. 8 years). The weight ranged from 3.4 to 8. 1 kg (mean, 5. 8 ± 2. 1 kg). Before surgery, all patients had apnea and cyanosis at resting state, and their resting SpO2 ranged from 65% to 85%. The heart/chest ratio calculated on X-ray film was from 0. 62 to 0. 77. Electrocardiograph (ECG) of the patients showed sinus rhythm, right atrium enlargement and right ventricle hypertrophy. Echocardiography revealed moderate to severe pulmonary hypertension and various degree of tricuspid reflux Under general anesthesia and cardiopulmonary bypass, direct anastomosis between the top of the left atrium and the common pulmonary venous trunk were performed via superior approach. Results The cardiopulmonary bypass time was 68. 7 ± 15. 3 min and the aortic cross-clamping time was 47. 6 ± 14. 7 min. The patients were followed up for 5 months to 3 years (mean, 18 months). No death after surgery was noted. Five patients had atrial arrhythmia (19. 1%)in the early postoperative stage. One patient had mild anastomotic stenosis, Two neonates had delayed sternum closure. Two patients had diaphragm paralysis, one of which underwent diaphragm placation.All of the patients except 2 had satisfactory cardiac function. Conclusions Correction of supracardiac total anomalous pulmonary venous connection via superior approach is safe and effective in neonates and infants.     

经心包斜窦行扩大吻合口心上型全肺静脉异位引流矫治术

目的 探讨经心包斜窦入路行心上型全肺静脉异位引流(TAPVC)矫治术,利用垂直静脉延长共同静脉切口,从而扩大共同静脉与左心房的吻合口.方法 2007年12月至2009年1月,我院共收治27例TAPVC患儿在体外循环下行畸形矫治手术,其中男17例,女10例;年龄2 d至13岁;体重2.3～21 kg,平均(5.16±3.49)kg;其中心上型TAPVC 11例,占40.7%;心下型1例,占3.7%;心内型12例,占44.4%;混合型3例,占11.1%.在全部接受手术的11例心上型TAPVC患儿中,10例采用经心包斜窦人路行扩大吻合口的矫治技术,其中7例同时采用无内膜接触缝合技术.此10例患儿中,男7例,女3例;年龄2 d至13岁;体重2.4～21 kg,中位数为6.0 kg.术后行心脏超声随访,随访时间为1～14个月,中位数为7个月,常规随访时间点分别设在出院前、术后3个月、术后半年及术后1年.手术选择心包斜窦入路,沿长轴横向剖开共同静脉,并将此切口上延至垂直静脉的心包返折处,使用7-0 PDS缝线将左心房后壁切口与共同静脉切口或其周边的心包组织吻合.结果 1例患儿因凝血功能障碍于术后第二天死亡;另1例患儿由于家庭原因于术后第五天放弃治疗死亡,其他8例患儿均存活.在8例存活患儿中,1例患儿因术前并发双侧肺实变,另1例患儿因合并重度肺动脉高压,术后机械辅助呼吸超过7 d,其他患儿均无并发症发生.至目前为止,心脏超声随访提示:全部存活患儿肺静脉回流通畅,血流速度介于0.65～1.2 m/s之间.结论 在治疗心上型TAPVC时,选择心包斜窦入路不仅可以获得良好的术野,还可以利用垂直静脉扩大吻合口内径,同时方便采用无内膜接触缝合技术,以预防术后肺静脉梗阻的发生.但本技术路线尚有待大宗病例的远期随访进行验证.     

左房顶径路矫治低体质量儿心上型完全性肺静脉异位引流12例

目的 探讨左房顶径路矫治低体质量儿心上型完全性肺静脉异位引流(TAPVC)的疗效.方法 选择本院2009年3月-2011年4月,低体质量TAPVC患儿12例.其中男7例,女5例;年龄10个月～12岁[(5.22±1.25)岁];体质量8.4～ 15.0(10.4±3.1) kg.患儿均在全麻、中低温体外循环下经左房顶径路完成矫治手术.正中切口纵劈胸骨入胸,经横窦,上腔静脉与升主动脉之间显露共同肺静脉干及左心房,将其侧侧吻合.体外循环时间52～102(67.2±14.2) min,其中主动脉阻断时间39 ～75(45.3±12.5) min.结果 术后早期(30 d内)无死亡,其中低心排出量综合征5例、心房扑动1例、交界性心律1例、肺部感染5例,均经治愈出院.11例随访1～3a,无死亡,其中出现交界性心律Ⅰ例,余均为窦性心律;随访超声心动图示吻合口无压力阶差10例,轻度狭窄1例.结论 在治疗心上型TAPVC时,选择左房顶径路治疗低体质量儿心上型TAPVC临床效果满意.     

完全性肺静脉异位连接的外科治疗

目的 总结10例完全性肺静脉异位连接的外科治疗经验。方法 全组均在全身麻醉中度低温体外循环下进行手术，6例心上型患儿早期1例经后径法矫治，其他5例中3例经右心房切口径路矫治；另2例采用心上法矫治；4例心内型1例采用自体心包片将冠状静脉窦口经扩大的房间隔缺损隔入左心房，后3例用5-0 Prolene线连续缝合房间隔粗糙边缘后再矫治。结果 无手术死亡，发生心律失常3例，一过性肺水肿2例，均经治疗痊愈。全组随访4个月～5年，心功能正常。结论 提高手术成功率和防止术后并发症的关键为：术中吻合口要足够大，扩大左心房容积及术后及时处理心律失常、肺水肿和低心排血量。心内型矫治时消除房间隔粗糙面可防止术后肺静脉梗阻。     

完全性肺静脉异位引流的诊断与治疗

目的探讨完全性肺静脉异位引流(TAPVC)的外科诊断与治疗。方法本组TAPVC患儿18例。年龄8个月~10岁;体质量10 kg以下3例。心上型14例,心内型4例。全组均在中度低温体外循环下将完全异位引流的肺静脉,直接或通过房间隔缺损隔入左房。结果手术死亡1例(5.6%)。随访14例,无死亡病例,远期效果满意。结论正确的术前诊断、手术技术改进和加强术后并发症防治,是TAPVC外科治疗取得满意疗效保证。     

心内型完全性肺静脉异位引流的解剖特征及手术矫治

目的 分析心内型完全性肺静脉异位引流(TAPVC)的解剖特征及手术矫治对策.方法 1981年4月～2006年12月手术治疗心内型TAPVC 25例(男17例,女8例),年龄26 d～14岁,平均(2.56±3.56)岁;体重3.3～21.0 kg,平均(6.85±10.06)kg.依据Darling分型,分为ⅡA及ⅡB两个亚型.所有患儿行超声心动图及心导管检查确诊,均在全麻低温体外循环下行矫治术.结果 所有患儿治愈出院.主要并发症为低心排出量综合征、肺水肿2例.随访3个月～26年,心功能均达到Ⅰ级,无肺静脉回流梗阻发生.结论 心内型TAPVC确诊后宜尽早手术,成功的关键在于术前、术中对其解剖特征准确判定及合适的心房分隔.     

混合型完全肺静脉异位引流外科治疗1例

完全肺静脉异位引流是一种少见而复杂的先天性心脏病,国内报道混合型肺静脉异位引流的矫治均为散发病例,缺乏大样本矫治结果支持。作者近年来收治2例混合型完全肺静脉异位引流患儿,疗效满意。     

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   

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.     

Bibliometric data: a disaster for many non-American biomedical journals

Bibliometric data published by the Institute of Scientific Information in Philadelphia (ISI), and which was previously discussed in Acta Paediatrica , has increasingly been used despite all the relevant and severe criticism that has been raised against this method of evaluating individual research results and grading scientific journals. It is obvious that the present trend regarding the use of bibliometric data as a basis for priorities and funding of research and for the promotion of individual scientists favours American-oriented research projects at the expense of those that are based on concepts of predominantly European relevance.

Conclusion: For the future of non-American research, it is important that no single super-power, i.e. the USA, should dominate scientific priorities. The condition for efficient European competition is that European Centres with high levels of competence for creative research and training of scientists from all over the world are established. In addition, it is important that the results of European research are published in prestigious European journals, as was the situation before World War II.     

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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.