几种胆道重建术治疗先天性胆总管囊肿的疗效评价

目的　评价先天性胆总管囊肿 (CCC)几种胆道重建术疗效。方法　CCC 38例均行囊肿切除 ,其中行肝总管十二指肠吻合术 6例 ,肝总管空肠Roux en Y吻合术 2 0例 ,空肠间置肝总管十二肠吻合术 9例 ,阑尾间置肝总管十二肠吻合术 3例。术后随访 6个月～ 6年。结果　获得随访 34例 ,其中肝总管十二指肠吻合术 5例 ,并胆管炎、钡餐造影钡剂反流各 2例 ;肝总管空肠Roux en Y吻合术 19例 ,钡餐造影钡剂反流 1例 ;空肠间置肝总管十二指肠吻合术 8例 ,吻合口狭窄伴肝内胆管扩张、胆瘘各 1例 ;阑尾代胆道 2例 ,并胆管炎 1例。结论　CCC术后远期疗效与术式选择、抗反流机制的建立密切相关 ,肝总管空肠Roux en Y吻合术伴抗反流术式 ,是囊肿切除术后重建胆道的理想术式     

胆总管囊肿切除不同胆道重建术的远期疗效评价

目的分析和评价先天性胆总管囊肿切除后采用不同胆道重建手术方式的远期治疗效果。方法对1985-2000年所行121例胆总管囊肿切除、胆道重建手术治疗的患儿资料进行总结和长期随访观察，胆道重建手术包括单纯肝管空肠Roux-Y吻合27例、肝管十二指肠吻合黏膜乳头成形24例、回盲部肠段间置14例和肝管空肠Roux-Y吻合加曾氏防反流瓣56例四种术式。结果91例获得远期随访，平均时间11．7g。2例发生恶变，出现反复发作胆管炎13例(单纯吻合组7例、黏膜乳头成形组5例和加防反流瓣组1例)、吻合口狭窄8例和肝门胆管结石5例，11例再手术后症状消失。结论根治切除囊肿是治疗本病的基础，肝门大口肝管空肠Roux-Y吻合加抗反流瓣术是胆道重建手术可选择的最佳术式。     

肝总管空肠Roux-y吻合术治疗婴儿胆总管囊肿

目的 介绍肝总管空肠Roux—y吻合术治疗新生儿及小婴儿先天性胆总管囊肿的体会。方法16例3个月以下婴儿及新生儿先天性胆总管囊肿患儿采用囊肿切除、空肠Roux—y吻合代胆道术，平均空肠代胆道肠袢45cm，全部病例在空肠胆枝袢做套叠式粘膜肌瓣，肝总管空肠吻合口及胆枝空肠吻合口用4—0可吸收缝线做单层端侧吻合。结果16例全部存活，1例术后发生胆漏，经保守引流治疗后痊愈，无一例肠漏，12例术前有黄疸的术后均明显消退，8例术前解陶土色大便，术后第1—3天就有胆汁样大便解出，随访12例，随访时间1—8年，临床观察无胆管炎发生，营养及生长指标良好。结论先天性胆总管囊肿行囊肿切除、胆道重建术作为标准手术已无争议，本组16例3个月以下婴儿行肝总管空肠Roux—y吻合加套叠式粘膜肌瓣抗返流，经临床观察及随访，远期手术效果好，未发生返流性胆管炎，无生长发育障碍。先天性胆总管囊肿经早期发现应早期行囊肿切除。而肝管空肠Roux—y吻合附加抗返流套叠式粘膜肌瓣术，是一种较为理想的术式。     

先天性胆总管囊肿28例治疗体会

目的 探讨先天性胆总管囊肿的治疗体会。方法 回顾分析1990年到2001年28例先天性胆总管囊肿的治疗,其中男10例,女18例,囊肿十二指肠吻合6例,囊肿外引流3例,囊肿切除肝管空肠Roux—Y吻合15例,囊肿切除空肠间置肝管十二指肠吻合4例。 结果 先天性胆总管囊肿应行根治性囊肿切除,肝总管空肠Roux—Y型吻合术或肝管空肠问置式并加人工抗反流措施。 结论 先天性胆总管囊肿应行根治性切除,并需注意近、远期并发症的防治。     

先天性胆总管囊肿63例报告

目的总结先天性胆总管囊肿的诊断与治疗经验。方法回顾1994年～2006年我院收治的63例患儿的病例资料,对其诊断、手术方式及手术后近、远期并发症进行分析。结果本组入院前后均经B超、CT、MRCP等检查,诊断符合率分别为88.9%、80.0%、90.0%。其中46例行囊肿切除 肝总管空肠Roux-en-Y吻合术,6例行囊肿切除 肝总管十二指肠吻合术,6例行囊肿内引流术。术后近期并发症:伤口出血5例,胆汁漏7例,胆系感染2例,吻合口梗阻1例。远期并发症:返流性胆管炎8例.肠梗阻4例,胆系结石2例。结论B超检查是诊断先天性胆总管囊肿的首选方法,囊肿切除 肝总管空肠Roux-en-Y吻合术是一种较好的治疗方法。     

先天性胆总管囊肿囊肿切除空肠间置与肝总管空肠吻合的远期随访与评价

目的 研究先天性胆这囊肿囊肿切除空肠间置与肝总管空肠Ｒｏｕｘ－Ｙ吻合重建胆管的远期随访结果与疗效评价。方法 对２种常用的胆管重建术：空肠间置术（２０例）和肝总管空肠Ｒｏｕｘ－Ｙ吻合术（３０例）进行３￣１０年随访。结果 获得随访４４例,其中短段空肠间置伴抗掺流瓣术１４例,肝总管空肠Ｒｏｕｘ－Ｙ吻合短段桥袢伴抗反流瓣术１８例均无反流性胆管炎,钡餐检查剂反流,而短段空肠间置不伴抗反流瓣４例中１例,肝总管     

先天性胆总管囊肿的诊治体会

目的 通过对47例先天性胆总管囊肿诊疗的回顾性分析提出理想的手术方法。方法 全部病例采用囊肿切除加胆肠吻合术重建胆道。结果 随访46例，其中24例采用短段空肠间置，同时加抗返流术式者20例；22例行Roux—Y肝总管空肠吻合术，同时加抗返流术式17例，均疗效满意，而加抗返流术式者疗效更优。结论 先天性胆总管囊肿的诊疗，应明确囊肿的分型及胆胰解剖，采用囊肿彻底切除，再行胆肠重建时应加抗返流术式。     

胆总管囊肿切除及T管引流应用的改进

目的 介绍胆总管囊肿广泛切除，留置直管引流的手术方法。方法 囊肿广泛切除，重建胆道肝总管空肠Roux—Y吻合，直接在吻合口下2～3cm肠壁戳口插入细直管，并呈隧道样包埋4～5cm一段。结果 本组16例术后造影吻合口通畅。结论 采用直管引流能防止囊肿切除胆道重建术后吻合口狭窄的并发症，并能通过造影了解胆管扩张情况。     

先天性胆总管囊肿64例的外科治疗

目的对先天性胆总管囊肿的外科治疗进行讨论。 方法 回顾性分析先天性胆总管囊肿64例,均经手术治疗。其中囊肿切除48例(Group I),总肝管空肠端端Roux—en—y式吻合16例(Group Ia),端侧吻合、胆道置管引流32例(Group Ib),囊肿同空肠Roux—en—y式吻合16例(GroupII)。结果Group II死亡1例,并发症率Group II 68.75％、Group Ia 25％、Group Ib 3.13％,本组总并发症率是20.31％,死亡率1.56％,远期治愈率为98.44％。 结论外科手术是本病唯一有效的治疗手段,为防止癌变、逆行感染、出血等,宜行囊肿切除,总肝管-空肠Roux—en—y式吻合胆道重建,总肝管-空肠端-侧吻合,置管胆道引流术式操作简便,安全可靠,并发症少,宜推广使用。     

重视腹腔镜技术治疗复合型胆管囊肿手术规划与术中处理

先天性胆管囊肿是一种胆总管单独或联合肝内胆管扩张的结构发育畸形,多伴有胆胰管合流异常,最常表现为胆总管囊状或梭形扩张,累及肝内胆管囊状扩张或副肝管畸形则被称为复合型胆管囊肿。随着微创技术的进步,腹腔镜胆总管囊肿切除、胆道重建手术在国内广泛开展,同时也开始应用到复合型胆管囊肿肝门胆管畸形的治疗中,但如果术中处理不当,容易发生血管误伤、胆肠吻合引流不畅等情况,继而导致反复胆管炎发作、肝内胆管结石等并发症,应予以重视。     

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.     

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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.