法乐四联症患儿心肌细胞和红细胞内外钙的变化

为了解法乐四联症（ＴＯＦ）患儿术前心肌舒张功能障碍的原因，采用钙荧光指示剂（Ｆｕｒａ－２）法和原子吸收分光光度（ＡＡＳ）法测定了１２例ＴＯＦ患儿心肌细胞、红细胞内外钙的变化。结果发现：ＴＯＦ术前心肌细胞游离钙（ＭｙｏＣａｉ）（３４７±８５ｎｍｏｌ／Ｌ）较先天性心脏病（简称先心病）组（１２２±１３ｎｍｏｌ／Ｌ）明显升高；红细胞游离钙（ＥｒｙＣａｉ）和红细胞总钙（ＥｒｙＣａｔ）（１．８９±０．５０Ｆ３３５／Ｆ３８５，２１．２±２．９μｍｏｌ／Ｌ红细胞）较先心病组（１．４７±０．０８Ｆ３３５／Ｆ３８５，１３．１±８．７μｍｏｌ／Ｌ红细胞）和健康组亦明显升高，而全血游离钙（ＢＣａｉ）和红细胞钠泵、钙泵活性明显下降。术后除钙泵活性外，ＢＣａｉ、ＥｒｙＣａｉ、ＥｒｙＣａｔ和红细胞钠泵活性均恢复正常。提示，钙内流增多和膜泵活性下降导致ＴＯＦ患儿心肌细胞和红细胞内存在过多钙积聚，可能是其术前舒张功能障碍的重要原因之一。     

支气管肺炎合并心力衰竭的发病机理研究

目的探讨支气管肺炎合并心力衰竭（简称心衰）的发病机理。方法对１５例肺炎合并心力衰竭患儿和１７例肺炎患儿及１７例正常健康小儿应用超声心动图检查测定左室收缩末室壁应力、射血分数（ＥＦ）、缩短分数（ＦＳ）和心率矫正之左室周径平均缩短速度（ｍＶｃｆｃ）；测定血管紧张素Ⅱ（ＡⅡ）和红细胞内游离钙水平。结果左室收缩末室壁应力：支气管肺炎合并心衰组（４９±６ｇ／ｃｍ２）较肺炎组（４２±９ｇ／ｃｍ２）和正常对照组（４１±８ｇ／ｃｍ２）增加；ＡⅡ：支气管肺炎合并心衰组（３６６±１６０ｎｇ／Ｌ）较肺炎组（５６±１６ｎｇ／Ｌ）和正常组（３８±８ｎｇ／Ｌ）增高；红细胞内游离钙水平：支气管肺炎合并心衰组Ｆ３３５／Ｆ３８５（１．９０±０．２８）和肺炎组Ｆ３３５／Ｆ３８５（１．８６±０．２６）较正常组Ｆ３３５／Ｆ３８５（１．６６±０．２４）增加；ＥＦ、ＦＳ和ｍＶｃｆｃ无改变。结论支气管肺炎合并心衰无心肌收缩力下降，而ＡⅡ增加、心脏后负荷增加。它们在支气管肺炎合并心衰的发病中起到重要作用。     

尼莫地平对法洛四联症患儿心肌细胞内钙运转及心功能的影响

目的　研究尼莫地平对法洛四联症（ＴＯＦ）患儿心肌细胞钙转运和心功能的影响。方法　２７ 例ＴＯＦ分为对照组（１２例）和治疗组〔１５ 例,尼莫地平１．０～１．５ｍ ｇ／（ｋｇ·ｄ）×１４ｄ〕。测定治疗前后心肌细胞内外钙浓度和心功能。结果　用尼莫地平后治疗组红细胞游离钙和总钙浓度明显降低,钠泵活性升高,而钙泵活性无变化;心肌细胞游离钙（２０４±３３ｎｍ ｏｌ／Ｌ）较对照组（３４７±８５ｎｍ ｏｌ／Ｌ）明显降低（Ｐ＜ ０．０１）;心肌细胞线粒体和内质网肿胀明显减轻或消失,线粒体密度升高;心脏指数和左右心室充盈功能明显改善。结论　尼莫地平可明显减轻ＴＯＦ患儿心肌细胞内外的钙超载,同时亦可改善心功能     

危重病儿甲状腺激素T3,rT3变化的临床意义

目的探讨危重病患儿甲状腺激素的变化及其临床意义。方法危重症患儿共４５例，包括重症肺炎、病毒性脑炎、格林巴利综合征，颅内出血、病毒性心肌炎、食物中毒等。２０例健康儿童为对照组。用美国ＰＡＣＫＡＲＤ公司生产的γ免疫自动计数器测定危重病儿和健康儿童血中Ｔ３及ｒＴ３含量。结果２０例健康儿童和４５例危重病患儿血中Ｔ３分别为（２．８８±０．５２）ｎｍｏｌ／Ｌ和（１．４０±０．７１）ｎｍｏｌ／Ｌ（ｔ＝１３．９８，Ｐ＜０．０１），ｒＴ３分别（为０．７２±０．０８）ｎｍｏｌ／Ｌ和（１．１９±０．７３）ｎｍｏｌ／Ｌ（ｔ＝４．３６，Ｐ＜０．０１），Ｔ３／ｒＴ３分别为２．７４±０．８５和１．６１±０．７９（ｔ＝６．３８，Ｐ＜０．０１）．４例临终状态病儿Ｔ３和ｒＴ３均值分别为０．８６和１．４８ｎｍｏｌ／Ｌ，Ｔ３／ｒＴ３为０．６３。４５例患儿，治愈３７例，好转４例，死亡４例。结论危重病患儿血中Ｔ３、ｒＴ３及Ｔ３／ｒＴ３均显著低于健康儿童．４例临终状态病儿血中Ｔ３、ｒＴ３及Ｔ３／ｒＴ３更低。提示甲状腺激素降低的程度与病情严重程度呈正相关。     

碱性成纤维细胞生长因子对胎鼠宫内窘迫脑损害的疗效观察

目的探讨母鼠应用碱性成纤维细胞生长因子（ｂＦＧＦ）是否对胎鼠宫内窘迫脑损害有保护作用。方法将５３只胎龄为２０天的ＳＤ大鼠胎鼠随机分为五组，即正常对照组（Ｃ组）１１只，窘迫对照组（Ｅ组）１０只，ｂＦＧＦ治疗组Ⅰ（Ｄ１组）１０只，ｂＦＧＦ治疗Ⅱ组（Ｄ２组）１０只，ｂＦＧＦ治疗Ⅲ组（Ｄ３组）１２只。在不同时间内静脉注射ｂＦＧＦ，监测静脉注射ｂＦＧＦ后胎鼠脑细胞内外钙、钠、钾含量的变化。结果在钳夹血管前２０分钟静脉注射ｂＦＧＦ后胎鼠脑细胞胞游离钙离子浓度为４７５±９１ｎｍｏｌ／Ｌ；再灌注同时静脉注射ｂＦＧＦ后游离钙离子浓度为４７４±４９ｎｍｏｌ／Ｌ。表明ｂＦＧＦ干预组均明显高于正常对照组（３１５±８７ｎｍｏｌ／Ｌ）（Ｐ＜００１），但亦均低于窘迫对照组（５５２±９４ｎｍｏｌ／Ｌ）（Ｐ＜００５）；同时这两组的脑组织总钙、总钠及总钾含量亦有不同程度的下降。结论无论是在窘迫前还是在缺血再灌注同时静脉注射ｂＦＧＦ，ｂＦＧＦ均可减轻宫内窘迫胎鼠脑细胞钙超载，减轻脑水肿，保护缺氧脑细胞；提示对产妇或窒息新生儿应用ｂＦＧＦ可能有助于减轻窒息新生儿脑损伤     

休克患儿磷脂酶A_2及细胞因子作用研究

目的探讨血清磷脂酶Ａ２（ＰＬＡ２）及细胞因子在休克发生发展中的作用及意义。方法采用ＥＬＩＳＡ法检测１６例休克患儿及２０例健康儿童血清ＰＬＡ２、肿瘤坏死因子（ＴＮＦ－ａ）及白细胞介素（ＩＬ～６）含量,并作临床脏器功能监测、血乳酸、血糖、动脉血气分析及血小板计数。结果休克患儿血清ＰＬＡ２（０．８９±０．６３ｍｐ／Ｌ）、ＴＮＦ－ａ（０、６３±０．２５ｍｇ／Ｌ）及ＩＬ－６（６．８４±１９７ｍｇ／Ｌ）浓度均较正常对照组（０．１７±０．０２ｍｇ／Ｌ、０．０８±０．０１ｍｇ／Ｌ、２．３２±０．６２ｍｇ／Ｌ）明显升高（Ｐ＜０．００１）;多器官功能衰竭组（ＭＳＯＦ）上述３项指标（ＰＬＡ２１．１７±０．７０ｍｇ／Ｌ、ＴＮＦ－ａ０．９１±０．２７ｍｐ／Ｌ、ＩＬ－６７．７０±１．４０ｍｇ／Ｌ）均显著高于单器官功能衰竭组（ＳＯＦ）（ＰＬＡ２０．４８±０．０７ｍｇ／Ｌ、ＴＮＦ－ａ０．４７±０．０５ｍｇ／Ｌ、ＩＬ－６５．５５±０．７０ｍｇ／Ｌ）,死亡组明显高于治愈组。结论ＰＬＡ２水平与病情轻重有关,可作为早期预测ＭＳＯＦ发生的参数、评估治疗效果及预后。选择性投用ＰＬＡ２抑制剂可能成为治疗休克的有效方法。     

哮喘豚鼠血红素氧合酶-1与内源性一氧化碳的相关性

目的　探讨血红素氧合酶１（ＨＯ１） 和内源性一氧化碳（ＣＯ） 在哮喘发病机制中的作用及相互关系。方法　利用哮喘豚鼠模型及药物预防,用分光光度法检测豚鼠血一氧化碳血红蛋白（ＣＯＨｂ） 和血清及肺组织匀浆上清液ＨＯ１ 活性水平,放射免疫竞争抑制法检测血浆ｃＧＭＰ。结果　哮喘组全血ＣＯＨｂ （９．２ ±１．４） ％ ;ＨＯ１ 活性：血清（１１３．３±１７．７） ｎｍｏｌ／（Ｌ·ｈ）、肺组织（５５．５±５．６） ｎｍｏｌ／（ｍｇ·ｈ） ;血清ｃＧＭＰ（１．５９ ±０．３０） ｐｍｏｌ／ｍｇ,分别与地塞米松预防组和正常组比较差异有非常显著意义（ｔ＝ １１．５１ ～２２．４７,Ｐ＜ ０．０１）。地塞米松预防组与正常组比较,差异亦有非常显著意义（ 除ｃＧＭＰ外,Ｐ＜０．０１） 。血清与肺组织的ＨＯ１ 活性水平呈正相关,且分别与血ＣＯＨｂ 和ｃＧＭＰ 含量亦呈正相关（ｒ＝０．８６～０．９７, Ｐ＜０．０１） 。结论　哮喘时豚鼠体内ＨＯ１ 活性、内源性ＣＯ 和ｃＧＭＰ水平同时升高,三者在哮喘发病机制中具有重要的作用和必然的联系。     

β-葡萄糖醛酸苷酶在母乳性黄疸发病中的作用

目的探讨β葡萄糖醛酸苷酶（βＧＤ）在母乳性黄疸发病中的作用。方法采用酶学比色法对４７例母乳性黄疸患儿及６０例正常新生儿粪便、血清及其母乳中βＧＤ活性浓度进行测定，并分析母乳性黄疸患儿βＧＤ活性浓度与血胆红素浓度的相关性。结果母乳性黄疸患儿母乳及粪便βＧＤ活性浓度分别为（１３６±０３０）Ｕ／Ｌ及（１０７±０３０）Ｕ／Ｌ，较正常新生儿［（０７５±０３３）Ｕ／Ｌ及（０５０±０２８）Ｕ／Ｌ］明显增高，二者之间差异有非常显著意义。患儿母乳及粪便βＧＤ活性浓度与血胆红素浓度呈明显正相关。黄疸消退期母乳及粪便βＧＤ活性浓度［（１１１±０３２）Ｕ／Ｌ及（０７２±０２６）Ｕ／Ｌ］较高峰期［（１３６±０３０）Ｕ／Ｌ及（１０７±０１０）Ｕ／Ｌ］明显降低，二者之间差异有非常显著意义。结论母乳及粪便βＧＤ活性浓度增高不仅与母乳性黄疸的发生有关，而且与其严重程度及病程经过密切相关。     

柯萨奇B3病毒对小鼠离体心肌细胞力学和收缩蛋白分子的影响

目的探讨病毒性心肌炎时病毒直接损伤心肌细胞的发病机理。方法培养新生ＢＡＬＢ／ｃ小鼠的离体心肌细胞，加柯萨奇Ｂ３病毒，造成病毒对心肌细胞直接损伤的细胞模型，检测心肌细胞力学和心肌收缩蛋白分子———心肌肌凝蛋白的α重链（αＭＨＣ），β重链（βＭＨＣ）的基因表达。结果柯萨奇Ｂ３病毒对体外培养的心肌细胞有高度的易感性，并造成收缩力下降，其中感染２４小时的心肌细胞收缩力减弱比１２小时更为明显；感染后的心肌细胞收缩蛋白分子发生了改变，表现为αＭＨＣ含量降低（由０．１５±０．０２减少到０．１３±０．０６），而βＭＨＣ含量增加（由０．０８７±０．００８增加到０．１１１±０．００９）。结论病毒直接损伤心肌细胞，使心肌细胞收缩蛋白分子的结构发生了改变，从而造成心肌细胞收缩力的下降     

哮喘儿童发病与血液一氧化氮、肿瘤坏死因子变化的研究

检测哮喘患儿血浆亚硝酸根／硝酸根（ＮＯ２－／ＮＯ３－）和血清肿瘤坏死因子α（ＴＮＦα）含量，并分析其相关关系。结果：正常对照组ＴＮＦα、ＮＯ２－／ＮＯ３－含量分别为（１６４．８５±３１．０８）ｎｇ／Ｌ和（２９．５４±１４．４３）μｍｏｌ／Ｌ；哮喘发作组分别为（１９８．７６±４２．０６）ｎｇ／Ｌ和（４６．６７±１９．１）μｍｏｌ／Ｌ，明显高于对照组（Ｐ＜０．０１），呈显著正相关（Ｐ＜０．０１）；哮喘缓解组为（１９２．４１±３９．５）ｎｇ／Ｌ和（３２．４±１４．９３）μｍｏｌ／Ｌ，ＴＮＦα高于对照组（Ｐ＜０．０５），ＮＯ２－／ＮＯ３－低于发作组（Ｐ＜０．０１）。提示ＴＮＦα和一氧化氮（ＮＯ）可能参与哮喘发作期气道炎症的形成，ＴＮＦα在缓解期的慢性炎症持续中具有重要意义     

Experience with heart transplantation in children

Between March 1981 and March 1986, 200 orthotopic heart transplantations were performed at the University of Pittsburgh. Fourteen of those procedures were carried out in children 2 to 16 years of age. Two children received combined liver and heart transplants; one because of familial hypercholesterolemia with associated ischemic heart disease, and the other because of dilated cardiomyopathy associated with intrahepatic biliary atresia. Eight patients had dilated cardiomyopathy, and two had myocarditis. Two had heart transplantations for congenital heart disease: one had multiple muscular ventricular septal defects repaired in infancy and had an associated cardiomyopathy, and the other developed a cardiomyopathic ventricle from a congenital right coronary artery to right atrial fistula. Chronic immune suppression consisted 0.2 to 0.5 mg/kg/d of prednisone and 5 to 50 mg/kg/d cyclosporine, with the addition of antithymocyte globulin for unresolved moderate or severe acute rejection. There were three early postoperative deaths: one from intracranial bleeding, one from Pseudomonas mediastinitis, and one from ischemic injury to transplanted organs. Early postoperative complications included reversible renal failure, hypertension, and seizures. Late problems were related to allograft rejection and side effects of cyclosporine and corticosteroids. Significant rejection episodes occurred in all patients surviving longer than 2 weeks, with seven requiring antithymocyte globulin. Two patients died 8 months following transplantation of severe acute and chronic rejection; another patient required retransplantation for ischemic cardiomyopathy resulting from chronic rejection but subsequently died of recurring rejection 3 months after the second transplantation.(ABSTRACT TRUNCATED AT 250 WORDS)     

新生儿青紫型先天性心脏病围术期心力衰竭的紧急治疗

对于青紫型先天性心脏病（简称先心病）的新生儿来说，围术期是极特殊的应激阶段。除了心脏畸形对血流动力学的影响外，患儿还受到心导管、心脏手术、麻醉和体外循环等的干扰影响。心脏术后重建血流动力学、心脏功能重新调整、体液酸碱和电解质失衡、内源性和外源性儿茶酚胺升高使病情更复杂，需在细致的治疗下度过危险期。新生儿先心病围术期心力衰竭的急救重点是积极生命支持和保持动脉导管开放。其他抢救措施包括保持通气、纠正代谢性酸中毒、补充血容量、应用正性肌力药物等增加心输出量和改善组织灌注，同时注意纠正低血糖和监控感染。     

Developmental delay in infants with congenital heart disease. Correlation with hypoxemia and congestive heart failure

The mental and motor development of 173 infants with congenital heart disease was assessed by means of the Bayley Scales of Infant Development and clinical neurological examinations. The relationship between age, sex, congestive heart failure, hypoxemia, hospitalization, and test results was evaluated. The presence of congestive heart failure was found to be significantly associated with both mental and motor development delay. Hypoxemia and hospitalization were associated with delayed motor development. Developmental delay could be recognized as early as 2 months of age.     

Cheyne-stokes respiration in children with heart failure

Cheyne-Stokes respiration (CSA-CSR) is a form of central sleep apnea characterized by alternating periods of hyperventilation and central apneas or hypopneas. CSA-CSR develops following a cardiac insult resulting in a compensatory increase in sympathetic activity, which in susceptible patients causes hyperventilation and destabilizes respiratory control. The physiological changes that occur in CSA-CSR include hyperventilation, a reduced blood gas buffering capacity, and circulatory delay. In adults, 25% to 50% of patients with heart failure are reported to have CSA-CSR. The development of CSA-CSR in this group of patients is considered a poor prognostic sign. The prevalence, progression, and treatment outcomes of CSA-CSR in children remain unclear with only 11 children being described in the literature. The lack of data is possibly not due to the paucity of children with severe heart failure and CSA-CSR but because they may be under-recognized, compounded by the absence of routine polysomnographic assessment of children with moderate to severe heart failure. Building on much broader experience in the diagnosis and management of CSA-CSR in adult sleep medicine and our limited experience in a pediatric quaternary center, this paper will discuss the prevalence of CSA-CSR, its’ treatment options, outcomes in children, and the potential future direction for research in this understudied area of pediatric sleep medicine.     

Employment in adults with congenital heart disease

OBJECTIVE: To evaluate job participation, career-related problems, and actual job problems in adults with complex congenital heart disease (CHD) compared with adults with mild CHD and reference groups. DESIGN: Cross-sectional study. SETTING: Patients were randomly selected from the archives of the Department of Pediatric Cardiology, Leiden University Medical Center, Leiden, the Netherlands. PATIENTS AND MAIN OUTCOME MEASURES: In total, 76 patients with complex CHD and 80 with mild CHD (age range, 17-32 years) completed a self-reported questionnaire on employment and handicaps, with reference data available (response rate, 70%). RESULTS: In the study groups, 45 (59%) of 76 patients with complex CHD had a paid job compared with 61 (76%) of 80 patients with mild CHD. Patients older than 25 years with complex CHD had significantly lower job participation (64%) than the general population (83%). Multiple logistic regression showed that type of CHD and level of education were significantly and independently related to job participation (odds ratio, 4.8; 99% confidence interval, 1.2-19.6; and odds ratio, 4.7; 99% confidence interval, 1.3-17.2, respectively). Of the 76 patients with complex CHD, 42 (55%) experienced disease-related career problems, in contrast to only 1 patient with mild CHD. Both CHD groups had more job-related mobility handicaps than did the reference group. However, in the mild CHD group, handicaps could be attributed to additional noncardiac diseases. CONCLUSIONS: Patients with complex CHD have reduced job participation compared with patients with mild CHD and the general population. Many receive disability benefits or experience career problems or job handicaps. Career counseling focusing on physical abilities and level of education may help prevent or reduce these job-related problems.     

Developmental outcome of patients with hypoplastic left heart syndrome treated with heart transplantation

OBJECTIVE: To describe the outcome of children treated for hypoplastic left heart syndrome (HLHS) with heart transplantation. STUDY DESIGN: We evaluated outcomes in 26 children treated for HLHS in a single center; 13 children were evaluated with the Bayley Scales of Infant Development, Child Behavior Checklist (CBCL), and Vineland Adaptive Behavior Scales (VABS), and 13 were seen after 36 months of age and were evaluated with the Wechsler Preschool and Primary Scale of Intelligence, CBCL, and VABS at 36 to 72 months or the Wechsler Intelligence Scale for Children-III, CBCL, and VABS for those older than 72 months of age. RESULTS: Bayley Scales of Infant Development results revealed a median Mental Developmental Index of 88 (range <50 to 102) and a Psychomotor Developmental Index of 86.5 (<50 to 113), both significantly lower than expected in the general population. Intelligence quotient results on either the Wechsler Preschool and Primary Scale of Intelligence or Wechsler Intelligence Scale for Children-III were also significantly lower than expected, with a mean verbal score of 90.5 +/- 12.4, performance score of 88.9 +/- 14.5, and full scale score of 88.5 +/- 13.0. On the Vineland scales, 39% scored >1 SD below the mean on measures of daily living scales, 22% on the socialization subscale, 48% on the communication subscale, and 52% on the adaptive behavior scale. CONCLUSIONS: In this small population of children treated for HLHS with heart transplantation, both cognitive deficits and adaptive/behavioral abnormalities are described. Early identification with appropriate referral for services could potentially enhance the outcomes for these children.     

A heart with three arterial trunks (tritruncal heart)

Summary The case is described of a 4 ?-year-old girl with a previously unreported malformation: a heart with three arterial trunks, aorta, main pulmonary artery, and an intermediate vessel, called “intermediate trunk” because it was situated between the aorta and the main pulmonary artery. Each of these three arteries had a semilunar valve at its origin, and their lumens were completely separated from each other. The pulmonary trunk arose from the right ventricle, while the aorta and the intermediate trunk arose from the left ventricle. The intermediate trunk continued as the right pulmonary artery. The pulmonary trunk continued as the left pulmonary artery. The outflow tracts of both ventricles were normal. There were therefore three arterial trunks arising from a heart with two outflow tracts. Our hypothesis on the embryopathogenesis of this case is that there was double septation of the primitive arterial trunk and the aortic sac, without involvement of the conus. In addition, there was a malalignment between the truncal and conal septa. This entity, however rare, should be considered in the differential diagnosis of absent or anomalous right pulmonary artery. Supported by the Centro de Cooperación Iberoamericano, Madrid, Spain