心外管道Fontan手术治疗复杂先天性心脏病

目的探讨心外管道Fontan手术方法治疗小儿复杂先天性心脏病的早期效果。方法9例复杂性先天性心脏病,男5例,女4例,年龄3~9岁。其中单心室5例,三尖瓣闭缩、右室双出口各2例。均在体外循环心脏不停跳下行心外管道Fontan手术。结果全组患儿无死亡,经皮血氧饱和度>95%,无心律失常,但均有明显胸腔积液表现。结论体外循环下心脏不停跳心外管道Fontan手术近期效果满意。     

婴幼儿肺炎合并先天性心脏病治疗和分析

目的 总结10 kg以下婴幼儿肺炎合并先天性心脏病、心力衰竭治疗经验.方法 本院2005年至2006年住院患儿共54例,男25例,女29例,年龄3个月～2岁,平均年龄(1.0±0.4)岁,体重5～10 kg,病种有VSD+PH、ASD+PS、VSD+ASD+PH、VSD+PDA、TOF、DORV等.入院后根据药敏情况选用合适抗生素,及强心利尿,扩血管等综合治疗.结果 所有病例经合理治疗后,均好转出院,无一例死亡.结论 对于患有先天性心脏病的婴幼儿应注意心功能情况,积极综合治疗,为手术作好准备,尽早手术.     

高频振荡通气在小儿先天性心脏病术后的应用

目的 探讨高频振荡通气(HFOV)在小儿先天性心脏病术后的应用效果.方法 2007年6月-2008年6月阜外心血管病医院小儿心脏中心实施心脏手术患儿1957例,其中实施HFOV 13例.男8例,女5例;年龄(13.4±15.4)个月;体质量4.2～12.5 kg.其中法洛四联症4例,大动脉转位3例(其中校正性大动脉转位1例),完全性肺静脉畸形引流、完全性心内膜垫缺损、肺动脉闭锁各2例.除1例肺动脉闭锁行体肺分流术外,其余手术均在全麻低温体外循环下进行.术后常规呼吸机辅助呼吸.采用SPSS 11.5软件进行统计学分析.结果 换HFOV前常频呼吸机辅助时间为(15.70±20.33)d,HFOV辅助时间为(10.0±7.5)d.高频通气早期可明显改善通气、换气功能,13例患儿中,存活5例,存活率38.4%.死亡病例中,6例死于肺实变,死于肾衰竭、肺出血各1例.7例并发气胸.结论 HFOV在先天性心脏病患儿术后的应用,早期可获得满意通气换气功能;但病死率仍很高;远期效果差者,HFOV可作为过渡手段.     

10公斤以下低体重婴幼儿先天性心脏病的外科治疗

目的：探讨10 kg以下低体重婴幼儿先天性心脏病的外科治疗问题。方法：自2000年1月至2007年12月,外科治疗10 kg以下低体重婴幼儿先天性心脏病105例,体重3.5~10 kg,年龄2个月至3岁,其中合并中度以上肺动脉高压和/或反复肺部感染史56例,复杂先天性心脏病35例。中度低温体外循环下手术88例,常温非体外循环心脏跳动下手术14例,深低温停循环选择性脑保护下手术3例;根治手术101例,姑息性手术4例。结果：住院死亡5例（4.8%）,存活100例,随访2月至3年,根治手术的97例中,室间隔缺损少量残余分流2例,无其他并发症和死亡,均生长发育良好,心功能I级;姑息手术的3例症状明显减轻。结论：随着围术期管理、麻醉、体外循环和外科技术的提高,10 kg以下低体重婴幼儿先天性心脏病外科治疗是安全的,临床效果良好。     

婴幼儿先天性心脏病合并气管狭窄的基层诊治策略

目的初步探讨婴幼儿先天性心脏病合并气管狭窄的基层诊治策略。方法回顾性分析泉州市儿童医院2014年5月至2015年11月间收治的25例先天性心脏病合并气管狭窄患儿的临床资料,对先天性心脏病并发或合并分支支气管轻中度狭窄的患儿采用术后早期拔管策略,合并严重气管狭窄的患儿建议转入上级医院接受进一步治疗,并对治疗结局进行评估。结果 18例合并轻中度气管狭窄的简单型先天性心脏病(室间隔缺损,房间隔缺损或动脉导管未闭等)患儿在我院接受先天性心脏病手术,术后均顺利撤离呼吸机; 7例先天性心脏病合并严重气管狭窄的患儿未在我院接受治疗,其中2例转入上级医院接受先天性心脏病联合气管狭窄手术,3例未及时接受手术而死亡。结论对于绝大多数合并节段性轻中度气道狭窄的简单型先天性心脏病婴幼儿,可采取保守治疗策略;而对于合并较为严重气管狭窄的患儿,在基层医院接受手术治疗存在较大风险,应尽早转上级医院接受心脏手术等一系列外科治疗。     

婴幼儿先天性心脏病的外科治疗

１１３例婴幼儿先天性心脏病患儿接受手术治疗．手术病死率２．７％，术后并发症发生率１３．３％，无后遗症发生．本文重点讨论婴幼儿先天性心脏病的手术时机，麻醉及体外循环方法，手术方法，术后呼吸系统和循环系统的处理原则．     

新生儿重症先天性心脏病术后腹膜透析

近年来随着先天性心脏病诊治水平的不断提高，新生儿期先心病手术量逐年增多，且多为急诊手术，从而增加了手术及术后的不确定因素。一些复杂重症先心病患儿手术后，由于新生儿各器官系统发育尚不成熟，以及长时间体外循环的影响，易并发严重的低心排伴容量超负荷、急性肾功能衰竭，是术后新生儿死亡的主要原因。新生儿单位体重的腹膜面积相对较大，因此其腹膜透析的效果比儿童和成人好，及时有效的腹膜透析可以纠治以上并发症，降低病死率。2003年10月～2004年10月我中心共收治新生儿先心病28例，均在体外循环下行手术治疗。其中5例术后给予腹膜透析治疗。现将其总结报告如下。     

当今小儿先天性心脏病诊治的几个问题

当今先天性心脏病诊治的方向始终是预防、早期诊断和治疗.经过30多年的努力,我国手术治疗先天性心脏病的医疗单位已达500余个,年手术能力5～6万例.新生儿期和复杂心脏病已在多中心广泛开展.为加快我国先天性心脏病的诊治步伐,笔者提出几个问题与同道商讨.     

先天性心脏病术后心律失常诊治经验

目的　探讨先天性心脏病术后心律失常的诊断与治疗。方法　对 1 37例先天性心脏病术后心律失常的发生率、发病因素、治疗与转归进行总结分析。结果　 1 37例先天性心脏病患儿均经体外循环下直视心脏手术 ,手术后有 5 6例发生 89例次心律失常 ,发生率 4 0 9% ,其中激动起源异常 31例次 ,以室上性心律失常多见 ,各型传导阻滞 5 8例次 ,以完全性右束支传导阻滞最多。结论　病因除手术因素外 ,以心功能不全、洋地黄中毒和电解质紊乱为主。除室内传导阻滞随访后未见恢复外 ,其他病例通过病因或抗心律失常治疗多数痊愈 ,有效率分别为 91 7%和 83 3%。     

婴儿及新生儿先心病心内直视手术208例

目的　探讨在婴儿、新生儿年龄段先天性心脏病心内直视手术的可行性及疗效评估。方法　对 2 0 8例先心病患儿实施了心内直视手术 ;手术年龄 3d～ 12个月 ,平均 (7.4 1± 3.6 7)个月 ,其中新生儿 17例 ,1～ 3个月 19例 ,3～ 6个月 4 1例 ,6～ 9个月 6 7例 ,9～ 12个月 6 4例。体重 3～ 11kg,平均 (7.4 6± 3.39)kg。男 135例 ,女 73例 ,手术病种 :室间隔缺损合并重度肺动脉高压类 15 5例(74 .5 % ) ,其余为D TGA、PA(IVS)、TAPVC等青紫复杂性先心病。结果　全组手术死亡 12例 ,病死率为 5 .9% ,年龄小、畸形复杂矫治不完全 ,术后低心排 (5例 ) ,呼吸道病变 (4例 )、术后顽固出血 (3例 )是导致死亡的主要因素。结论　手术治疗是拯救婴儿、新生儿高危复杂先心病的积极、有效的手段。随着麻醉、体外循环、监护技术、手术器械材料的进一步改进 ,手术存活率有望进一步提高     

Role of nutrients in the regulation of in vivo protein metabolism in humans

Nutrients regulate protein metabolism both in an acute fashion and on a long-term basis. The ingestion of meals is associated with a dramatic switch from an overall catabolic state to a state of net protein anabolism. The acute response of protein metabolism to meal ingestion is mediated, in part, by an increase in insulin secretion, itself a consequence of glucose absorption. Whereas insulin may primarily suppress rates of proteolysis, amino acids are responsible for the stimulation of protein synthesis that follows food intake. In the long run, the effects of nutrition on protein metabolism depend on the energy supply, the source of the energy (carbohydrate versus fat) and dietary protein intake. Finally, specific amino acids, such as glutamine, may play an additional role as protein anabolic agents.     

氨磷汀在儿童肿瘤化疗中的应用

目的研究氨磷汀在儿童肿瘤化疗中的作用及不良反应。方法随机选择肿瘤32例患儿,采用自身对照,观察患儿在同一化疗方案中使用氨磷汀辅助治疗后黏膜损伤、骨髓抑制时间、发热持续时间、抗生素及刺激因子应用时间及氨磷汀的不良反应。结果氨磷汀辅助治疗组发生黏膜溃疡4例,明显低于对照组9例;中性粒细胞<0.5×109/L持续时间缩短,血小板<5×109/L持续时间缩短;发热持续时间、抗生素和刺激因子应用时间缩短。两组化疗过程中均未出现低体温、皮疹、低血压等情况,化疗后肾功能、心电图均正常;对肝功能影响程度相似;氨磷汀组低钙血症尤其是低钙性抽搐发生率高。结论氨磷汀能够有效保护正常组织细胞,不良反应较低,可在儿童肿瘤患者化疗中应用。     

Determinants of pregnancy in adolescents in Nepal

Objective : Social factors and prevalent norms in the community determine the proportion of teenage pregnancy in the community. In the light of high risk associated with teenage pregnancy, the socio-cultural determinants, which influence the conception among teenagers in Nepal, need to be understood. These determinants may be modified by suitable interventions to reduce teenage pregnancy. Aim of this study was to examine the socio-cultural determinants of teenage pregnancy in eastern Nepal.Methods : A case-control study design was selected for comparing the education, economic status, family support and freedom towards conception among teenagers as compared to higher age group women.Result : Seventy adolescent pregnant women were compared with seventy primigravida women in the 20 to 29 years age group. The teenage pregnant women were less educated, had poor economic background, more likely to have accidental pregnancies as compared to the other group and more likely to have love marriages. Husbands were more likely to decide about continuation of pregnancy. They had less psychological and social support from the family.Conclusion : Marriage at young age and pregnancy during teens are associated with less social acceptance and poor support in the family.     

Diagnosis of Chlamydia pneumoniae in community-acquired pneumonia in children in Chile

The incidence of Chlamydia pneumoniae in community-acquired pneumonia in children was studied prospectively in 112 children aged 1 mo to 14 y. Diagnosis of C. pneumoniae was performed by polymerase chain reaction (PCR) on nasopharyngeal aspirates and serology by the microimmunofluorescence test on a single serum specimen. Three (2.7%) cases of pneumonia due to this agent were diagnosed by both PCR and serology. C. pneumoniae was not found in any of 62 children below 5 y of age. In the age group 5-8 y, only 1/30 (3%) was found positive, and in the age group 9-14 y, C. pneumoniae was diagnosed in 2/20 (10%) children. Conclusion: Although the number of enrolled patients is small, and the diagnostic techniques used may have some limitations, the results of this study suggest that C. pneumoniae plays a minor role in the aetiology of pneumonia in children less than 9 y of age in our country. However, it should be considered as a potential agent in pneumonia in older children.     

Efficacy of amphotericin B in multi-drug resistant kala-azar in children in first decade of life

Fifty children in the first decade of life, and suffering from multiple drug resistant kala-azar, confirmed by demonstration of amastigotes in aspirates of bone marrow or spleen were treated with amphotericin B in gradually increasing dosage to a total dose of 20 mg/kg. All patients had classical features of severe kala-azar, and had taken more than one course of antimony and pentamidine, and three patients had taken one additional course of ketoconazole besides many courses of antimony and pentamidine. The clinical response started just after first infusion in 8 patients, and the patients became afebrile. By 5th infusion, all looked better and 18 patients became afebrile. By 15th infusion all patients were afebrile and cheerful. Their spleens became smaller and body weights and total white cell counts increased. Forty eight patients had parasitological cure at the end of treatment, and only 2 patients required an additional 5 infusions for parasitological cure. All patients were ultimately cured. No one relapsed within six months of follow up. All patients had shivering, rigor and rise of temperature on the day of infusion, which could be minimized with prior administration of low dose of hydrocortisone, but could not be eliminated. Eighteen patients had loose motions during treatment, while 14 patients had decrease in appetite which improved quickly when the treatment was over. Fourteen patients had transient rise of blood urea, in six patients serum creatinine also increased and 16 patients had a minor fall in serum potassium. It is concluded that amphotericin B is a very effective drug for multidrug resistant kala-azar in children, and should be used without hesitation in all such cases in children, but under close medical supervision.     

Role of nutrients in the regulation of in vivo protein metabolism in humans

Nutrients regulate protein metabolism both in an acute fashion and on a long-term basis. The ingestion of meals is associated with a dramatic switch from an overall catabolic state to a state of net protein anabolism. The acute response of protein metabolism to meal ingestion is mediated, in part, by an increase in insulin secretion, itself a consequence of glucose absorption. Whereas insulin may primarily suppress rates of proteolysis, amino acids are responsible for the stimulation of protein synthesis that follows food intake. In the long run, the effects of nutrition on protein metabolism depend on the energy supply, the source of the energy (carbohydrate versus fat) and dietary protein intake. Finally, specific amino acids, such as glutamine, may play an additional role as protein anabolic agents.     

他汀类药物在儿童家族性高胆固醇血症治疗中的研究进展

家族性高胆固醇血症(FH)是一种常染色体显性遗传的脂质代谢障碍疾病,由于FH早期就可引起动脉粥样硬化病变,增加患者严重心血管疾病的患病风险和死亡风险,因此目前越来越强调患者自儿童期就使用他汀类药物进行治疗,以改善远期预后。文章综述他汀类药物治疗儿童FH的必要性、有效性和安全性,同时也提出目前存在的一些问题和思考。     

皮质激素在癫痫治疗中的应用

皮质激素在癫痫中的应用已有近50a的历史，现已证明其对某些癫痫综合征如婴儿痉挛症、Landau—Kleffner综合征、Rassmussen综合征、具有睡眠中癫痫性电持续状态的癫痫综合征等具有肯定的治疗作用，其确切的作用机制尚不清楚。在皮质激素应用过程中应注意其不良反应。