儿童心脏自主神经功能检查方法

<正>随着临床诊断技术的发展,心脏自主神经功能检查方法也得到不断改进和完善,成为评价自主神经功能状态及对自主神经功能紊乱性疾病诊断和鉴别诊断的依据。本文就目前儿童常用的心脏自主神经功能检查方法作一介     

Congenital malformations in Shimla

Congenital malformations were studied over a period of five years in 10, 100 consecutive births including still births at Indira Gandhi Medical College, Shimla. Out of these, 180 babies had one or the other congenital malformations and the overall incidence was 1.78%. Amongst the 311 still born babies 47 had congenital malformations indicating that the incidence of congenital malformations was much higher in still born babies (15.1%) as compared to the live born babies (1.3%). The malformations involving the central nervous system were the commonest (40%) followed by musculoskeletal system (23.8%) while genitourinary system malformations were the least common and accounted for 3.8% of the cases. Incidence of congenital malformations was the highest in mothers over 35 years of age and gravida four and more. The incidence was 2.8% in both the groups; the incidence of congenital malformation was more in babies weighing < 2500 gms and was 2.6%.     

The stress of birth enhances in vitro spontaneous and IL-8-induced neutrophil chemotaxis in the human newborn

The birth process induces fetal stress. Stress has profound effects on the immune system, also by acting on the trafficking of leukocytes, a process in which adhesion and chemotaxis are primordial and critical events for the development of effective antimicrobial defenses. The newborn is rapidly challenged by a microflora at the epithelia linings and therefore depending on early, innate immunity onset. The objective of the study was to investigate the immune response in cord blood from newborns in relation to different degrees of fetal stress, with focus on neutrophil chemotaxis. We analyzed in vitro transmigration ability of neutrophils and their CD11b expression, measured total white blood count (WBC) and the major leukocyte populations, interleukin (IL)-8, interferon (IFN)-gamma, and soluble E-Selectin, as well as relevant immuno-modulating hormones in infants born at term after Cesarean section prior to the start of labor (n = 55), normal vaginal delivery (n = 87), and assisted delivery (n = 26). Arterial pH and lactate were used as stress markers. We found that spontaneous and IL-8-induced transmigration ability of neutrophils from newborns after normal delivery was significantly higher compared with that of neutrophils from Cesarean section or from adults. With a progressive increase in fetal stress, there were significant elevations in total WBC, in particular neutrophils and monocytes, as well as an enhanced IL-8 and soluble E-Selectin level. Assisted delivery, associated with the highest degree of fetal stress in addition had an enhanced lymphocyte and monocytes count as well as an increased IFN-gamma level. There were significant direct correlations between neutrophils and monocytes, respectively, with cortisol, beta-endorphin, and prolactin. Interferon-gamma was directly related to dopamine, as well as to the lymphocyte and monocyte count. The setting of the HPA-axis at birth is a promoter of an alarm response and a surge of neuroendocrine immuno-modulating factors that enhances antimicrobial defenses of the newborn. We speculate that IL-8 induced by normal labor may be a priming factor for an increased neutrophil chemotaxis through the pre-activated endothelium of the fetus. Assisted delivery may trigger excessive recruitment of additional inflammatory cells and IFN-gamma release.     

神经内镜在小儿颅内囊液性病变治疗中的应用

目的 探讨神经内镜技术在小儿颅内囊液性疾病中的治疗应用,以及结合开颅术及分流术协同完成小儿颅内复杂畸形手术治疗的效果.方法 2004年2月至2009年2月,105例患儿接受内镜手术,年龄3个月至12岁.包括阻塞性脑积水53例,其中51例成功完成内镜下三脑室底造口术.脑内囊肿52例中有28例颞叶囊肿行镜下囊壁部分剥离术和脑池开放术,5例透明隔囊肿和5例侧脑室囊肿分别行囊肿-脑室造口术、3例鞍上及三脑室囊肿行囊肿剥离+三脑室底造口,幕下囊肿6例:其中内镜剥离1例,5例巨大囊肿合并脑积水者采取内镜结合分流或开颅显微镜下剥离术.幕上中线部位囊肿5例,囊肿部分剥离+开窗4例,1例多房打通结合囊肿腹腔分流术.结果 51例脑积水中,47例术后临床症状改善.52例颅内囊肿,术后随访2个月至3年,总体缩小率占98%,颞叶囊肿缩小率100%,11例术后有硬膜下积液,7例随访好转,4例术后4个月至1年出现慢性硬膜下血肿,行外引流术后治愈,1例合并中枢感染者放弃治疗.结论 内镜手术在小儿颅内囊液性疾病的治疗中效果良好,可优先选择.     

同源盒基因家族在神经系统中的研究进展

同源盒基因家族编码一类转录调节因子,能特异地结合并调控靶基因,控制胚胎发育及细胞增殖分化.该家族中的多个亚家族与神经系统的早期分化发育密切相关,它们的异常表达参与了神经系统疾病的发生发展.研究同源盒基因家族与神经系统的关系,有利于预防神经系统异常分化发育和疾病的发生,为探索治疗相关疾病提供新途径.     

�Һ-��ë���ϵͳ�ڶ�ͯ �����������е�����

呼吸道在呼吸过程中会持续遭受环境中有害气体和病原微生物侵袭的危险,对有害吸入物的及时清除需要依靠呼吸系统的防御机制。正常的气道黏液-纤毛转运系统对维护气道的防御功能有重要意义。许多复杂的防御机     

Intracranial tuberculosis in the Netherlands: Four paediatric cases

Abstract Since 1987 a resurgence of tuberculosis is occurring in the Netherlands in analogy to the situation in other industrialised countries. So far this has not been associated with an increased incidence of tuberculous meningitis, which is still a rare complication of the disease. Four cases of intracranial tuberculosis were recently diagnosed in our hospital. All children were from middle-class Dutch families with no identified risk for tuberculosis. An extensive contact identification search could identify a contact in two patients. Two children were in an advanced stage of disease before diagnosis. Skin tests were repeatedly negative in all cases which delayed treatment in two cases for almost 2 weeks. One patient developing a cerebral tuberculous focus during therapy responded well to a second course of corticosteroids. Three children developed severe neurological sequelae.Conclusion This report illustrates that suspicion of intracranial tuberculosis is warranted in the case of an ill-defined inflammatory neurological syndrome.     

血小板在脓毒症中的研究进展

脓毒症为感染致全身免疫失调的炎症反应综合征,血小板在脓毒症发生发展中起着重要作用。血小板可通过黏附、聚集、活化、脱颗粒作用与病原微生物相反应,以达到保护机体的作用。同时,脓毒症时血小板还可与中性粒细胞等免疫细胞相互作用,参与微血栓、炎症的形成。因此,血小板有抗微生物效应及与其他先天免疫细胞协作,形成复杂的血管内免疫防御...     

小儿泌尿生殖系横纹肌肉瘤的治疗

目的：评价小儿泌尿生殖系横纹肌肉瘤不同治疗方法的疗效。方法：回顾1972年1月－1994年12月收治的16例小儿泌尿生殖系模纹肌肉瘤临床资料，前期治疗以手术＋化疗为主，共治疗7例，其中Ⅲ期5例，Ⅳ期2例，后期治疗采用活检确诊＋冲击量化疗＋手术＋放疗的综合疗法，共治疗9例，其中Ⅲ期7例，Ⅳ期2例。结果：前期治疗术后5年仅存活1例，后期治疗术后5年存活3例，均为Ⅲ期患儿。结论：应用活检确诊＋冲击量化疗＋手术＋放疗的综合疗法可提高Ⅲ期泌尿生殖系横纹肌肉瘤的存活率。     

豚鼠围产期巨细胞病毒感染肝胆损伤模型的建立

Objective To establish a perinatal cytomegalovirus (CMV) induced hepatobiliary system injury model in guinea pigs. Methods Three experimental groups were designed as follows. (1) Prenatal group (Group P): Female guinea pigs on the 40th to 43rd gestational day were randomly allocated into 3 subgroups. Guinea pigs in group P1 and P2 accepted intraperitoneal injection of virus supernatant with the dose of 1 10 9 TCID per dam and saline respectively, while guinea pigs in group P3 served as blank control. Live-born pups were sacrificed within 24 hours, on day 10 or 20 after birth. Samples of livers, extrahepatic bile duct and blood were collected. Weight gain, clinical signs of hepa-tobiliary injury (I. E. , jaundice in non-fur-covered skin, acholic stools) and survival were recorded. (2) Neonatal group (Group N) : A subset of healthy pups accepted intraperitoneal injection of virus supernatant at the dose of 1 108 TCID per pup within 24 hours after birth. Samples were collected on day 10, 20, or 30 after birth. (3) Infantile Group (Group Ⅰ): Healthy pups were inoculated with a same dose of virus supernatant on day 10 after birth. Samples were harvested on day 10, 20 or 30 after inoculation. The level of total bilirubin (TB), direct bilirubin (DB), ALT and AST in blood samples was analyzed. Serial sections of the liver or extrahepatic bile duct fixed in formalin were stained with Hematoxylin and Eosin. Hybridization in situ was applied on frozen sections to detect the distribution of viral mRNA. Results Compared to those in the control group, TB, DB, ALT and AST levels in Pups infected prenatally were significantly higher within the first 10 days of life (P<0. 05), and de-creased to the normal level on 20th day of life. A few pups of P1 group got the signs of jaundice (ie. Acholic stools ), which were associated with increased TB and DB level. In the neonatal group, a higher AST level was observed on the 10th day post inoculation in pups but it returned to normal soon. Birth weight of pups infected prenatally was significantly lower than the saline injection group or the blank control group (P = 0. 0029, P = 0027, respectively). Significant growth retardation was noted after 20 days post inoculation, while not noted in the neonatal or infantile group. Histological analysis of the livers revealed a notable infil-tration of mononuclear cells in 63. 6% (14/22) of pups in the prenatal group. Inflammatory cells were mainly found in portal tract area. Interlohular portal tract damage and fihrosis together with bile ductular proliferation were noted. Balloon-like degeneration, necrosis and ductular cholestasis were found in liver tissues of a part of pups. Micro-abscesses were occasionally observed. Pathological changes were also found in pups inoculated within the first day of life (3/8), but not as severe as those in the prenatal group. No significant pathologic change was found in the infantile group. The gpCMV rnRNA signal was found in endothelia, epithelia and portal tract stoma, while no signal was found on hepatoeytes. Positive rate of gpCMV was 17. 27% (4/22) in pups of Group P1, significantly lower than that in pups of Group N or Ⅰ (55% vs 50%, respectively). Conclusions Intraperitoneal injection with non-enhanced gpCMV on the late gestational days of pregnant guinea pigs or within 24h after birth of neonatal pups could induce inflammation and injury in the hepatobiliary system.     

小儿原发性胃肠道非霍奇金淋巴瘤

目的对我院8例原发性胃肠道非霍奇金淋巴瘤的临床资料进行分析,讨论其临床病理特点及手术对该病治疗的意义。方法收集1989年1月至2006年5月在我院8例原发性胃肠道非霍奇金淋巴瘤的病例,结合文献对其临床表现、诊断、治疗及预后等进行总结分析。结果8例均为经病理、免疫组化等检查确诊的胃肠道非霍奇金淋巴瘤,其中胃非霍奇金淋巴瘤2例,肠6例(小肠4例、回肓部2例)。临床分期:Ⅱ期6例,Ⅲ期1例,Ⅱ期～Ⅲ期1例,病理免疫类型:8例均为B细胞性非霍奇金淋巴瘤。8例均行手术治疗,术后辅以联合化疗,方案为COP或CHOP。6例可随访,1例失随访,1例死亡,随访率75%。结论小儿胃肠道非霍奇金淋巴瘤以B细胞性最多见,回盲部及近回盲部小肠最多见,直肠最少;本病术前诊断率较低,完整手术切除是原发性胃肠道非霍奇金淋巴瘤的治疗关键,不能完整切除的病例,活检术值得推荐,术后辅以化(放)疗及综合性治疗措施,可以提高患儿生存期。     

三维电生理标测系统在儿童心律失常导管消融中的应用

目的 探讨三维标测系统(Carto和Ensite)在指导儿童心律失常导管消融中的安全性、适应证和优越性.方法 ①在Carto指导下房性心动过速(atrial tachycardia,AT)消融8例;年龄(6.2±1.7)岁,体重(18.0±2.0)kg.②在Ensite指导下心律失常消融10例.其中应用Ensite Array系统指导消融8例,包括频发右室室性早搏(premature ventricular contractions,PVCs)6例,右房AT2例,年龄(11.3±1.2)岁,体重(40.0±5.0)kg;应用Ensite NavX系统指导消融2例,为B型预激综合征.结果 ①在Carto指导下8例AT患儿成功消融,其中"切口"性AT6例,左房耳AT和右房AT各1例;1例"切口"AT术后3个月复发,再次消融成功.②在Ensite Array指导下6例频发右PVCs和2例右房AT患儿成功消融,其中PVCs从术前(32 333±4509)个/24 h下降至0～4个/24 h;1例AT患儿1 d后出现另一种周期的AT.在Ensite Navx指导下成功消融2例B型预激综合征.随访6个月无复发.结论 Carto系统适合持续心律失常患儿的检测,而Ensite Array系统适合大于10岁、不持续右心心律失常患儿的检测;Ensite NavX可迅速建模,显示心内解剖结构.     

先天性小肠闭锁术后肠动力功能障碍的原因探讨

目的探讨小肠闭锁术后发生肠动力功能障碍的原因及手术切除病变肠管范围。方法对小肠闭锁手术切除小肠标本15例及对照组6例非肠道或神经系统疾病死亡的足月新生儿尸检小肠标本肠壁S-100蛋白、α-平滑肌肌动蛋白（α-SMA）和c-kit蛋白进行免疫组化研究。观察闭锁两端肠壁肠神经系统（ENS）、平滑肌和肠间质细胞（ICCs）病理改变及其分布范围，并行统计学处理。结果闭锁近端肠壁S-100、α-SMA和c—kit阳性表达明显低于对照组。随远离盲端，以上指标呈逐渐增加趋势。在闭锁近端16cm、远端4cm处，三者病变总体趋于正常。结论小肠闭锁两端肠壁与肠动力密切相关的ENS、平滑肌和ICCs均存在病变。是小肠闭锁术后发生肠道动力功能障碍的原因。在患儿小肠长度允许的情况下．切除闭锁近端肠管16cm以上，而远端切除4cm．可减少或避免术后肠动力功能障碍的发生。     

Cardiovascular drugs in children: Angiotensin-converting enzyme inhibitors

Summary Angiotensin-converting enzyme inhibitors are potent vasodilators acting by inhibition of production of the vasoconstrictor angiotensin II. In adults, they are used for treatment of systemic hypertension and congestive heart failure and investigated for treatment of primary pulmonary hypertension.In infants and children, saralasin and captopril were found to be useful in treatment of systemic arterial hypertension, especially when associated with high plasma renin activity. Captopril has failed in the treatment of congestive heart failure associated with complex congenital heart diseases and in most cases of primary pulmonary hypertension. It has a clear beneficial effect in coarctation of the aorta and may have such an effect in endomyocardial diseases and ventricular septal defect.In adults, serious side effects have limited the use of captopril. New converting enzyme inhibitors, devoid of a sulfhydryl group, are expected to have a better safety profile.     

幼年型类风湿关节炎中枢神经系统损害14例

目的 探讨幼年型类风湿关节炎（ＪＲＡ）发生中枢神经系统（ＣＮＳ）受损的临床特点,指导治疗,改善预后。方法 对１４例伴ＣＮＳ损害的ＪＲＡ患者的临床资料分析、总结并进行随访。结果 有ＣＮＳ损害的ＪＲＡ患者的临床资料分析、总结并进行随访。结果 有ＣＮＳ受损的ＪＲＡ患者均为全身型,７８％ＣＮＳＸ受损出现在ＪＲＡ病程１ａ以上。临床表现有：癫痫样发作、昏迷、偏瘫、头痛、嗜睡等。糖皮质激素治疗效果较好。结论 Ｊ     

The Relationship Between Manual Ability and Ambulation in Adolescents with Cerebral Palsy

ABSTRACT

This study examined the relationship between gross motor function and manual ability in 120 adolescents with cerebral palsy (CP) (15.2, SD 2.1 years, 59.8% male). Adolescents were evaluated using the Gross Motor Function Classification System (GMFCS) and the Manual Ability Classification System (MACS). A neurologist classified CP subtype. Most adolescents were ambulatory with or without utilization of aids (GMFCS level I: 35.0%, II: 30.0%, III: 5.8%, IV: 10.8%, and V: 18.3%). MACS levels were I: 34.2%, II: 25.8%, III: 16.7%, IV: 7.5%, and V: 15.8%. Correlations between GMFCS and MACS were strong in youth with quadriplegia (r = .89, p < .001), moderate in individuals with diplegia (r = .58, p = .01), but weakly associated for adolescents with hemiplegia (r = .24, p = .23). The findings provide evidence for maintaining a clinical distinction between spastic quadriplegia and spastic diplegia. Manual ability may not be congruent with mobility in adolescents with CP and should be specifically evaluated given its importance to daily life functioning.     

儿童髓母细胞瘤的显微手术治疗

目的 总结61例显微手术切除儿童髓母细胞瘤的经验，探讨儿童髓母细胞瘤手术治疗策略。方法 自1995～2002年同济医院对61例儿童髓母细胞瘤进行了显微手术治疗。其中男46例，女15例，年龄1～10岁，平均6．3岁。病程3～25周，平均6周。病变位于小脑半球1例，60例肿瘤均位于小脑蚓部。肿瘤1～3cm者7例，3～5cm者25例，大于5cm以上者27例。所有病例手术后通过影像学(CT和MRI检查)和生存质量的观察，随访1～7年(随访5年以上4例，2～4年46例，2年以内ll例)。半年、1年、3～5年分别复查CT或MRI，6例患儿因经济原因仅在手术后半年时，复查1次。结果 全组病例中，肿瘤全切55例，大部切除6例，手术死亡3例。病死率为4．9％(3／61)，未出现严重并发症。患儿生存质量得到提高。结论 掌握显微手术方法和手术中注意事项，有利于提高肿瘤切除程度及减少手术并发症，手术后必须辅助放疗，通过采取这些措施，将使儿童髓母细胞瘤获得良好预后。     

肛门直肠畸形人类及大鼠神经系统的并发症及神经支配研究进展

先天性肛门直肠畸形(anorectal malformations,ARM)是最常见的小儿消化道畸形,占消化道畸形的1/4.尽管长期以来ARM的手术方式得到不断改进,但长期随访发现仍有许多中高位ARM患儿术后存在不同程度的排便功能障碍.研究人员意识到术后肛门直肠功能不良取决于许多因素,骶尾椎畸形以及骶尾部脊髓发育不良已成为影响术后排便功能的重要因素.研究人员通过对人类胚胎以及动物实验研究发现,腰骶椎异常是ARM常见的伴发畸形,腰骶髓神经细胞存在减少的现象.人类和大鼠胚胎发育过程中涉及肛门直肠和盆底肌神经支配的相关因素繁多.该文介绍人类及大鼠肛门直肠畸形神经系统并发症以及神经支配的异常,总结对该病合并神经支配异常的认识和研究发展过程,并提出了关于肛门直肠畸形神经系统研究的新方向,探索改善肛门直肠畸形手术预后的新策略.     

��ͯ����Ĥĸϸ����

??Retinoblastoma??RB?? is the most common intraocular tumor in population??and represents 3% of pediatric cancers. The most common intimal sign of RB was "leucocoria". It develops in the retina as a primitive neuroectodermal tumor that affects development during gestation. It is initiated by mutation of the RB1 gene??which was the first described tumour-suppressor gene. It could be in one eye or both eyes. The treatment for RB included interventional treatment??intraocular conservation??chemotherapy and radiotherapy??and the long-term survival rate reached over 95%. However??with the deepening of understanding and improvement of economic conditions??the rate of visits for RB and spread of RB in the central nervous system has been increasing year by year. Understanding the diagnosis and comprehensive treatment of retinoblastoma and research progress at home and abroad are necessary to improving the eye-protection rate and quality of life in children with RB.