婴幼儿主动脉缩窄合并心内畸形的治疗策略

目的 总结婴幼儿主动脉缩窄(CoA)合并心内畸形的治疗经验.方法 2003年1月至2009年12月手术治疗21例CoA合并心内畸形患儿.15例患儿行左侧开胸矫治CoA,正中开胸修补心内畸形;6例正中开胸同时矫治CoA和心内畸形.结果 因低心排血量综合征和多器官功能衰竭死亡2例,病死率为9.5%.呼吸机应用7 h～13 d,ICU住院时间3～18 d.术后无神经系统并发症,超声检查无残余主动脉缩窄.结论 术前避免漏诊、尽早手术、选择正中切口一期根治、术后严防并发症是CoA矫治手术成功的关键.     

婴幼儿主动脉缩窄合并心内畸形的治疗策略

目的 总结婴幼儿主动脉缩窄(CoA)合并心内畸形的治疗经验.方法 2003年1月至2009年12月手术治疗21例CoA合并心内畸形患儿.15例患儿行左侧开胸矫治CoA,正中开胸修补心内畸形;6例正中开胸同时矫治CoA和心内畸形.结果 因低心排血量综合征和多器官功能衰竭死亡2例,病死率为9.5%.呼吸机应用7 h～13 d,ICU住院时间3～18 d.术后无神经系统并发症,超声检查无残余主动脉缩窄.结论 术前避免漏诊、尽早手术、选择正中切口一期根治、术后严防并发症是CoA矫治手术成功的关键.     

先天性心脏病围手术期气管狭窄的综合治疗

目的 通过分析先天性心脏病围手术期气管狭窄患儿的治疗及转归,探讨不同原因导致气管狭窄的治疗方法.方法 回顾性分析2012年1月至2015年10月收治的60例气管狭窄合并先天性心脏病患儿的临床资料.其中,男42例,女18例;年龄1～144个月,平均4个月;体重(6.65±3.40) kg.先天性心脏病合并先天性气管狭窄39例,继发性气管狭窄21例.结果 先天性气管狭窄合并先天性心脏病39例,实施先天性心脏病矫治+气管狭窄旷置28例,先天性心脏病矫治+气管成形术5例,先天性心脏病矫治+气管支架置入1例;术后远期死亡共计4例,放弃手术治疗死亡4例,放弃治疗门诊随诊1例.继发性气管狭窄21例,实施心脏畸形矫治后气管软化狭窄减轻,不需手术治疗.结论 先天性心脏病合并先天性气管狭窄的主要致病原因在于其完全性软骨环导致气管内径增速缓慢,气道梗阻严重,即使实施心脏手术也不能完全改善其气管狭窄症状,必要时需同时实施气道成形术.继发性气管狭窄的主要致病原因在于气管临近大血管的压迫,缓解血管环畸形后气管狭窄即刻缓解,疗效满意,无需实施气管成形术.     

婴儿及新生儿血管环并呼吸道压迫的诊断与治疗

目的探讨婴儿及新生儿血管环并呼吸道压迫的诊断与治疗方法。方法2004年6月-2009年12月广东省人民医院收治、经手术证实的血管环并呼吸道压迫婴儿及新生儿16例(男7例,女9例;年龄1 d～12个月),其中13例并心内畸形。患儿均行胸片、心脏超声及螺旋CT检查,其中9例行纤维支气管镜检查。15例于体外循环下行血管环松解术,1例行血管环松解术+心包补片扩大气管成形术。11例在术中同时治疗心内畸形。结果肺动脉吊带5例(其中1例并气管环),右位主动脉弓/左侧韧带或迷走左锁骨下动脉8例,双主动脉弓1例,无名动脉压迫综合征1例,肺动脉吊带、左位主动脉弓/迷走右锁骨下动脉形成2处血管环并气管环1例。超声心动图漏诊9例。16例CT检查均诊断为血管环。纤维支气管镜检查见气管受压。其中14例治愈或症状消失出院,2例并气管环者死亡。随访11例,其中5例术后仍间断有轻度喘息发作。结论婴儿及新生儿血管环并呼吸道压迫多表现为气促、喘鸣及呼吸困难,螺旋CT在该病诊治中具有重要作用。血管环松解术能使其呼吸道受压情况缓解,气管狭窄明显者应同时行气管成形术。     

肺动脉吊带的外科诊治

肺动脉吊带(pulmonary artery sling,PAS)是一种先天性心血管疾病,由于压迫气管和食管,常可造成严重的呼吸道梗阻或食道受压症状,一旦确诊即有手术指征.目前国内外对于PAS的外科治疗方案较多,包括单纯左肺动脉重建术、气管狭窄矫治术、气管球囊扩张和支架置入术等.通常依据气管直径和横截面积大小来决定是否同时行气管狭窄矫治术,虽然气管狭窄矫治手术方法较多,但尚无理想方法解除气管狭窄;对合并复杂心内畸形的PAS患儿应同时在体外循环下行心内畸形矫治.     

二次手术治疗肺动脉吊带矫治术后气管狭窄

目的探讨肺动脉吊带合并气管狭窄的治疗方法。方法分析2017年2月至2022年10月山东大学附属儿童医院收治的26例肺动脉吊带矫治术后因气管狭窄接受二次手术治疗的患儿资料。其中男14例, 女12例;中位年龄为31.3个月;中位体重为13.7 kg;两次手术间隔中位时间为12.5个月。1级狭窄3例, 2级狭窄15例, 3级狭窄8例。合并右位心3例, 合并左肺动脉狭窄或闭锁3例, 合并迷走左锁骨下动脉畸形及Kommerell憩室1例。患儿均采用Slide气管成形术加宽气道。术后定期复查电子支气管镜。非正态分布数据采用Wilcoxon符号秩检验(配对样本)。结果 26例患儿气管狭窄段长度中位数为4 cm, 范围为2.5～6.6 cm。本研究病例无手术死亡, 无住院期间死亡。术后呼吸机辅助中位通气时间为19.1 h。术后并发症包括吻合口漏合并胸骨哆开1例, 声带麻痹3例, 气管软化1例, 乳糜胸1例, 脑损伤2例。术后中位随访时间为7.0个月。活动耐力略差3例, 其余患儿无呼吸道症状。3例行左肺动脉加宽术的患儿术后定期复查心脏彩色多普勒超声检查提示左肺动脉血流通畅。3例声带麻痹患儿术后3个月症状...     

先天性主动脉缩窄29例临床分析

目的 总结我院心脏中心小儿主动脉缩窄的治疗、监护经验.方法 先天性主动脉阻塞性疾病患儿29例,包括主动脉缩窄28例,主动脉弓离断2例,主动脉双弓1例,单纯性主动脉缩窄3例,合并其他心内畸形23例.年龄15 d～6岁,5 kg以下患儿占40%.单纯主动脉阻塞采用侧开胸左后外侧第4肋间进胸,游离主动脉弓降部,结扎切断动脉导管,切除缩窄段,行端端吻合;对合并心内畸形和其他血管畸形的主动脉狭窄的患儿实施Ⅰ期手术,同时矫治心内畸形与血管畸形.结果 本组患儿29例,手术当日上下肢血压收缩压方面,下肢大于上肢者占68%,一致者(<5mm Hg)占21%,下肢小于上肢者占11%.1例因撤机困难放弃治疗;1例死亡.结论 单纯主动脉阻塞性疾病或合并其他心内畸形的患儿应早期手术治疗,Ⅰ期根治术安全、有效.     

先天性主动脉缩窄29例临床分析

目的 总结我院心脏中心小儿主动脉缩窄的治疗、监护经验.方法 先天性主动脉阻塞性疾病患儿29例,包括主动脉缩窄28例,主动脉弓离断2例,主动脉双弓1例,单纯性主动脉缩窄3例,合并其他心内畸形23例.年龄15 d～6岁,5 kg以下患儿占40%.单纯主动脉阻塞采用侧开胸左后外侧第4肋间进胸,游离主动脉弓降部,结扎切断动脉导管,切除缩窄段,行端端吻合;对合并心内畸形和其他血管畸形的主动脉狭窄的患儿实施Ⅰ期手术,同时矫治心内畸形与血管畸形.结果 本组患儿29例,手术当日上下肢血压收缩压方面,下肢大于上肢者占68%,一致者(<5mm Hg)占21%,下肢小于上肢者占11%.1例因撤机困难放弃治疗;1例死亡.结论 单纯主动脉阻塞性疾病或合并其他心内畸形的患儿应早期手术治疗,Ⅰ期根治术安全、有效.     

先天性主动脉缩窄29例临床分析

目的 总结我院心脏中心小儿主动脉缩窄的治疗、监护经验.方法 先天性主动脉阻塞性疾病患儿29例,包括主动脉缩窄28例,主动脉弓离断2例,主动脉双弓1例,单纯性主动脉缩窄3例,合并其他心内畸形23例.年龄15 d～6岁,5 kg以下患儿占40%.单纯主动脉阻塞采用侧开胸左后外侧第4肋间进胸,游离主动脉弓降部,结扎切断动脉导管,切除缩窄段,行端端吻合;对合并心内畸形和其他血管畸形的主动脉狭窄的患儿实施Ⅰ期手术,同时矫治心内畸形与血管畸形.结果 本组患儿29例,手术当日上下肢血压收缩压方面,下肢大于上肢者占68%,一致者(<5mm Hg)占21%,下肢小于上肢者占11%.1例因撤机困难放弃治疗;1例死亡.结论 单纯主动脉阻塞性疾病或合并其他心内畸形的患儿应早期手术治疗,Ⅰ期根治术安全、有效.     

纤维支气管镜在大血管疾病合并气道狭窄中的诊断价值

目的 评价纤维支气管镜(简称纤支镜)在先天性大血管病变合并气道狭窄中的诊断价值及安全性.方法回顾性分析2005年10月至2009年6月NICU收治的经纤支镜检查确诊气道狭窄的先天性大血管病变患儿34例,其中血管环10例,主动脉梗阻性病变24例,年龄6 d～11个月,体重2.2～8.7 kg[(4.6±1.4)kg];记录纤支镜发现的气道狭窄情况、CT及手术所见确诊的心血管畸形及气道狭窄情况,总结分析纤支镜检查发现的气道狭窄与心血管畸形的关系、气道狭窄的处理及转归.结果 所有病例均在NICU或手术过程中顺利完成纤支镜检查.(1)34例气道狭窄的首发症状为气促、喘鸣、反复肺部感染和呼吸机依赖.(2)10例血管环患儿均先经纤支镜检查,发现气道外压性狭窄,提示血管环,气道狭窄以气管下段为主;9例经CT、1例经手术证实为血管环,其中7例伴先天性气管狭窄.(3)24例主动脉病变中5例为术前CT提示气管狭窄,其中1例纤支镜提示气道狭窄,余19例在主动脉梗阻性畸形矫治术中、术后经纤支镜发现气道压迫狭窄.24例主动脉病变中21例有左主支气管狭窄,2例有先天性气管狭窄.纤支镜检查与CT检查对气管狭窄的诊断基本吻合.(4)34例纤支镜检查过程中2例出现一过性血氧饱和度下降,5例出现一过性心动过速,其余患儿无不良反应.结论 纤支镜检查在大血管畸形伴气道狭窄的诊断中起重要作用,具有准确、安全、方便、快捷的优点,但对气管狭窄的情况、气管周围结构和血管畸形的了解需结合CT检查.     

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.