产前超声诊断胎儿完全性大动脉转位

目的探讨产前超声对完全性大动脉转位的诊断价值。方法回顾分析7例胎儿完全性大动脉转位的产前超声图像。结果 7例胎儿完全性大动脉转位中,5例为单纯型,2例伴有室间隔缺损。6例四腔心切面正常。心室流出道切面示肺动脉起自左心室,主动脉起自右心室;主动脉和肺动脉根部相互交叉关系消失,二者平行走行。三血管气管切面仅见主动脉横弓与上腔静脉。结论心室流出道切面和三血管-气管切面是检查大动脉转位的主要切面,产前超声心动图是诊断胎儿大动脉转位有价值的方法。     

Anatomical correction of complete transposition of the great arteries and ventricular septal defect in infancy.

Two patients, aged 8 weeks and 5 years, with D transposition of great arteries and large ventricular septal defect were treated by transection of both aorta and pulmonary arteries and reattaching them to the appropriate ventricles. This included the origins of the coronary arteries. The ventricular septal defect was closed through a transverse ventriculotomy using a Dacron patch. The younger child was operated on as an emergency because of cyanosis and severe heart failure resistant to intensive medical treatment. The older child had had previous banding of the pulmonary artery at the age of 1 year. In both patients pulmonary artery pressure dropped to below half systemic pressure immediately after the operation. Postoperative progress was satisfactory with relief of cyanosis and heart failure. Early anatomical correction of transposition of the great arteries and ventricular septal defect is feasible and should play an important part in the management of these patients.     

Defining critical periods for itraconazole-induced cleft palate, limb defects and axial skeletal malformations in the mouse

The main aim of the study was to identify the critical periods of susceptibility for itraconazole-mediated teratogenesis in the mouse. Pregnant ICR (CD-1) mice received a single oral administration of itraconazole at 50 mg/kg b.w., 150 mg/kg b.w. or 250 mg/kg b.w. on gestation day 8, 9, 10, 11 or 12. Control animals were administered with vehicle on gestation days 8-12. The gestational outcome was evaluated near term, on gestation day 18. Treatment-related morphological findings, as they can be evaluated by external, visceral and skeletal examination, mainly included cleft palate, limb defects and axial skeletal malformations. Cleft palate and limb defects resulted after single exposures between gestation days 9 and 11, with a tendency toward maximal response on gestation day 10. Significant incidences of axial skeletal defects were seen exclusively on gestation days 8 and 9. Exposure on gestation day 12 did not yield significant teratogenic responses. Cleft palate was the most sensitive teratogenic response, being the only developmental lesion associated to exposure to itraconazole at 150 mg/kg b.w.     

WNT5A基因rs566926多态性与中国苏皖地区部分人群非综合征性唇腭裂的相关性

目的 探讨WNT5A基因rs566926位点多态性与中国苏皖地区部分人群非综合征性唇腭裂的相关研究.方法 选取100例非综合症性唇腭裂(NN2LP)患儿为实验组,100例正常儿童作为对照组,应用聚合酶链式反应-限制性片段长度多态性(PCR-RFLP)方法检测WNTSA基因rs566926多态性.采用病例对照研究方法进行统计分析并依据临床病理类型进行分类分析.结果 两组基因型频数分布有明显差异(P<0.05).结论 WNT5A基因rs566926位点多态性与中国苏皖地区部分人群NSCLP的发生有相关性,可能是该人群NSCLP发生的易感因素之一.     

BMP4基因rs17563位点多态性与中国华东地区部分人群非综合征性唇腭裂的关系

目的探讨骨形态发生蛋白4(BMP4)基因rs17563位点多态性与中国华东地区部分人群非综合征性唇腭裂(NSCL/P)的关系。方法收集65个唇裂核心家庭血样,分为患儿(患儿组)和患儿父母(父母组)组,另设正常儿童65例为对照组。对BMP4外显子4的rs17563位点突变碱基进行测序。比较患儿与正常儿童中的分布差异,比较患儿组与父母组的基因型和等位基因频率,并对核心家庭的数据进行单倍型相对风险分析(HRR),对含杂合子父母的核心家庭进行传递不平衡检验(TDT)。结果患儿组与对照组的基因型分布差异有统计学意义(P<0.05)。患儿组与父母组各位点基因型和等位基因分布差异无统计学意义(P>0.05),HRR结果和TDT结果无统计学意义(P>0.05)。结论 BMP4基因rs17563位点多态性与中国华东部分人群NSCL/P存在相关性。     

中国人群非综合征性唇腭裂与MTHFR基因C677T多态性

目的 探讨MTHFR基因C677T多态性与中国人群非综合征性唇腭裂（NSCL／P）发生的关系。方法 应用聚合酶链式反应一限制性片段长度多态性（PCR-RFLP）方法检测MTHFR基因C677T多态性,用病例对照方法进行统计分析。结果 计算TDT,X^2=0.02,P〉0.05;患儿和正常儿童等位基因频数比较,X^2=9．91,P〈0．05;有无家族史比较,差异无显著性。结论 MTHFR基因C677T位点多态与中国人群NSCL／P的发生相关。     

Ethnic differences in the incidence of cleft lip and/or cleft palate in Auckland, 1960-1976

The incidence of facial clefts among live births in the Auckland urban area has been studied for the years 1960-1976. After correction for ascertainment, the live-born incidence of non-syndromal cleft palate among Maoris was 1.867/1000, and among Europeans it was 0.643/1000. The incidences for non-syndromal cleft lip, with or without cleft palate, were 0.397/1000 for Maoris, and 1.195/1000 for Europeans. The sex ratio for cleft palate showed the usual female preponderance, although there was evidence of heterogeneity between the races. Cleft lip occurred more commonly among males. Birth rank, maternal age, and paternal age had no apparent effect on the incidences, and there were no significant secular or seasonal changes in incidence.     

三维多层面剖析新技术在胎儿腭显示及腭裂诊断中的价值研究

目的评价三维多层面剖析新技术在胎儿腭显示及腭裂诊断中的价值。方法对126例中晚孕胎儿应用侧角度照射技术采集容积数据,使用随机后处理软件对容积数据进行多层剖析,分别获取胎儿腭的经口裂、轴向、冠状及矢状切面等4组多层剖析图。产后及引产后追踪。结果 120例颜面正常的胎儿中98例成功采集到容积数据。经多层剖析后处理均能获得理想的4组多层剖析图。6例唇腭裂胎儿采集的容积数据经多层剖析后处理后能较好地显示腭裂的部位、类型、累及范围。结论三维多层剖析技术能获取胎儿腭的三正交平面及其它特殊切面的连续多层剖析图,可对原发腭及继发腭的完整性进行全面评估,对是否存在腭裂及腭裂部位、类型及累及范围作出明确诊断。为产前诊断腭裂提供了一项新的探查途径及诊断技术。     

A novel ETA-receptor antagonist, FR 139317, inhibits endothelin-induced contractions of guinea-pig pulmonary arteries, but not trachea.

1. The effects of a proposed endothelin-receptor antagonist, FR 139317, on the contraction induced by endothelin-1, endothelin-2 and endothelin-3, were analysed on isolated circular segments of pulmonary arteries and rings of trachea from the guinea-pig. 2. The pharmacological profiles of endothelin-1 and endothelin-2 were almost identical in the guinea-pig pulmonary artery, whereas endothelin-3 demonstrated a weaker and less potent contractile effect. The contractions induced by endothelin-1 and endotheliln-2 were competitively antagonized by FR 139317. Schild plot analysis revealed a straight line with a slope that did not differ from unity. The pA2 value was 6.65. In contrast, the endothelin-3 induced contractile response was unaffected by FR 139317. 3. In tracheal segments endothelin-1, endothelin-2 and endothelin-3 evoked contractions of similar magnitude and sensitivity. FR 139317 had no effect on the endothelin-induced contractions in tracheal segments. 4. In ring segments of pulmonary artery and trachea, potassium, noradrenaline and histamine caused concentration-dependent contractile effects. These contractions were not modified by FR 139317 in the concentration range 10(-7) to 3 x 10(-6)M. 5. FR 139317 seems to be a selective ETA-receptor antagonist which competitively antagonizes the endothelin-1- and endothelin-2-induced contractions of guinea-pig isolated pulmonary arteries. Thus, the guinea-pig pulmonary artery appears to be endowed with one receptor type (ETA) which is antagonized by FR 139317 and with another endothelin-receptor subtype which responds to endothelin-3, but is not antagonized by FR 139317.(ABSTRACT TRUNCATED AT 250 WORDS)     

Synergistic effects of peloruside A and laulimalide with taxoid site drugs, but not with each other, on tubulin assembly

Previous studies on the drug content of pelleted tubulin polymers suggest that peloruside A binds in the laulimalide site, which is distinct from the taxoid site. In a tubulin assembly system containing microtubule-associated proteins and GTP, however, peloruside A was significantly less active than laulimalide, inducing assembly in a manner that was most similar to sarcodictyins A and B. Because peloruside A thus far seems to be the only compound that mimics the action of laulimalide, we examined combinations of microtubule-stabilizing agents for synergistic effects on tubulin assembly. We found that peloruside A and laulimalide showed no synergism but that both compounds could act synergistically with a number of taxoid site agents [paclitaxel, epothilones A/B, discodermolide, dictyostatin, eleutherobin, the steroid derivative 17beta-acetoxy-2-ethoxy-6-oxo-B-homo-estra-1,3,5(10)-trien-3-ol, and cyclostreptin]. None of the taxoid site compounds showed any synergism with each other. From an initial study with peloruside A and cyclostreptin, we conclude that the synergism phenomenon derives, at least in part, from an apparent lowering of the tubulin critical concentration with drug combinations compared with single drugs. The apparent binding of peloruside A in the laulimalide site led us to attempt construction of a pharmacophore model based on superposition of an energy-minimized structure of peloruside A on the crystal structure of laulimalide. Although the different sizes of the macrocycles limited our ability to superimpose the two molecules, atom correspondences that were observed were consistent with the difficulty so far experienced in creation of fully active analogs of laulimalide.     

术前音乐视频干预对唇腭裂患儿修复术全麻苏醒期躁动的影响

目的 探讨术前音乐视频干预对唇腭裂患儿修复术全麻苏醒期躁动的临床效果.方法 采用随机对照试验设计,选择64例行唇腭裂修复术患儿分为观察组和对照组,两组均进行常规护理,观察组术前实施3d音乐视频干预.比较两组患儿苏醒前后心率、呼吸、血氧饱和度和躁动评分(PAED).结果 患儿复苏期苏醒后躁动发生率观察组低于对照组,观察组心率、呼吸、血氧饱和度、PAED与对照组差异有统计学意义(P＜0.05).结论 音乐视频干预可明显减轻唇腭裂修复术患儿麻醉苏醒期躁动的发生,有利于患儿病情恢复并减少术后意外事件发生.     

A new device for fixing the premaxilla with osteotomy in a child with complete bilateral cleft lip and palate in the mixed dentition period--a preliminary case report

In the treatment of a protruded premaxilla associated with complete bilateral cleft lip and palate, some patients require surgical setback of the premaxilla with osteotomy as functional and aesthetic treatment. No satisfactory surgical approaches have been established for the setback because it is usually difficult to fix the premaxilla after osteotomy in the mixed dentition period. This paper reports the new method for fixing the premaxilla with osteotomy in a child with a severely protruded premaxilla in the mixed dentition period. To fix the segment, we fabricated a device consisting of a palatal bite plate and an anterior tray before surgery. The space between the tray and the premaxilla was filled with soft resin during surgery so that the reposition of the premaxilla could be adjusted. This approach produced a favorable result. Our device has several advantages. It allows the adjustment of repositioning of the premaxilla during surgery. It can change the range of the adjustment according to the intended position of the premaxilla. It allows secure fixation of the segment. It is removable for tooth brushing and observation of bone union. Our device can be applied to many children similar to our patient reported in this paper.     

Effectiveness of cardiac glycosides in human myocardium with and without "downregulated" beta-adrenoceptors.

We investigated the "receptor-effector-coupling" in the beta-adrenoceptor- and the Na+, K(+)-ATPase-mediated systems in nonfailing hearts and terminally failing human myocardium from patients with cardiomyopathy. The density of beta-adrenoceptors in the failing human myocardium was significantly (p less than 0.01) lower as compared with nonfailing hearts, whereas the receptor density and affinity measured by [3H]ouabain binding (cardiac glycoside receptor) was not different in either group. The maximal inotropic response to isoprenaline was significantly reduced in papillary muscle strips from failing human hearts (2.1 +/- 0.5 mN) as compared with control hearts (8.0 +/- 1.0 mN; p less than 0.05). Ouabain remained effective in both groups (6.8 +/- 1.0 vs. 5.5 +/- 0.6 mN; NS). The positive inotropic response due to extracellular Ca2+ elevation (1.8-15 mM) was studied for comparison. Maximal Ca2+ effects were reduced by 30% in failing human myocardium (7.2 +/- 0.5 mN vs. 5.1 +/- 0.8 mN, p less than 0.05). Ouabain had effectiveness (95%) similar to that of Ca2+ in nonfailing and failing human cardiac muscle. It is concluded that treatment with cardiac glycosides may still be effective in end-stage heart failure with "downregulated" beta-adrenoceptors, as judged from these in vitro studies.     

主、肺动脉的超声观测诊断胎儿心脏大血管畸形的研究

目的 探讨主、肺动脉的超声观测作为产前筛选胎儿心脏大血管畸形主要线索的诊断价值。方法 对100例20～40周正常孕妇采用超声心动图测量主、肺动脉内径，并计算肺动脉内径和主动脉内径比值(PA／AO)。按照PA／AO的正常比值对2683例20周以上孕妇进行胎儿先天性心脏病(CHD)筛选。结果 （1）不同孕龄的胎儿其PA、AO内径随孕龄增加而增大，但是其PA／AO比值相对较为恒定，约为1．0～1．2。(2)多种胎儿心脏大血管畸形包括主动脉弓发育不良、主动脉狭窄、肺动脉狭窄、法乐氏四联征、主动脉缩窄等，其PA／AO比值明显异常。结论 胎儿PA／AO作为产前筛选胎儿CHD指标，尤其是胎儿大血管畸形，是一项实用的指标。     

Chronic hypoxia differentially alters the responses of pulmonary arteries and veins to endothelin-1 and other agents.

The effects of chronic hypoxia on the responses of rat large pulmonary arteries and veins to vasoactive agents have been examined. Endothelin-1-induced contractions of pulmonary arteries and pulmonary veins were reduced by chronic hypoxia. In contrast, chronic hypoxia augmented sarafotoxin 6c-induced contractile responses in pulmonary veins but not in pulmonary arteries. Chronic hypoxia augmented the constrictor effect of phenylephrine in pulmonary arteries, but not in pulmonary veins. The thromboxane receptor agonist, U46619 (9,11-dideoxy-9alpha,11alpha-epoxy-methanoprostaglandin++ + f2alpha) contracted pulmonary arteries and pulmonary veins, and although maximal responses were not altered in chronically hypoxic preparations, the EC50 value in pulmonary arteries was increased following chronic hypoxia. The relaxant effects of acetylcholine and isoprenaline on pulmonary arteries were potentiated by chronic hypoxia. In contrast, ionomycin-mediated relaxations of pulmonary arteries and pulmonary veins were reduced, while sodium nitroprusside-induced relaxation of pulmonary arteries and veins were not altered by chronic hypoxia. Previous studied have looked primarily at the effects of chronic hypoxia on pulmonary arteries. This data provides evidence that chronic hypoxia also causes selective changes in the reactivity of large pulmonary veins.     

Fortimicins A and B, new aminoglycoside antibiotics. IV. In vitro study of fortimicin A compared with other aminoglycosides.

The in vitro antimicrobial activity of fortimicin A, the most active member of the fortimicin complex, was compared with that of amikacin, gentamicin, sagamicin and tobramycin against 352 strains of Enterobacteriaceae and other medically significant organisms. Against most of these organisms fortimicin and amikacin had comparable levels of antimicrobial activity, generally slightly less than that of gentamicin, sagamicin or tobramycin. Fortimicin had relatively weak activity against Pseudomonas aeruginosa strains. Fortimicin shows many of the characteristics of other aminoglycoside antibiotics: (i) improved activity at alkaline pH, (ii) rapid, bactericidal action, (iii) reduced activity with increasing inoculum levels, and (iv) synergistic activity with penicillin against enterococci. The activity of fortimicin was compared to that of gentamicin, tobramycin and amikacin against a group of 95 naturally occurring, antibiotic-resistant Gram-negative bacilli other than Pseudomonas. The organisms were isolated from clinical sources and selected primarily for gentamicin resistance by the sensitivity disc test. Fortimicin showed excellent activity against this group of organisms. At a concentration of 6.2 mcg/ml, fortimicin inhibited the most strains (92.6%) followed by amikacin (90.5%), gentamicin (23.2%) and tobramycin (8.4%).