上海地区胆道闭锁筛查报告

目的探讨基于婴儿大便色卡的筛查方案在上海地区进行胆道闭锁筛查的可行性和有效性。方法于2015年1月1日至2016年10月31日向在上海四家产科医院出生的新生儿家长发放新生儿大便色卡并宣教使用方法。新生儿回家后,家长若发现存在黄疸且大便颜色异常,可直接联系胆道闭锁筛查中心进行进一步检查。若未联系,产科医院会于新生儿42 d回访时回收色卡,并按照筛查方案向本筛查中心传报疑似病例,由筛查中心追踪至诊断或排除胆道闭锁。所有确诊胆道闭锁患儿均需经胆道造影和/或胆道探查明确诊断。以家长在42 d回访前联系胆道闭锁筛查中心并被识别或在42 d回访时被产科医院识别并传报为胆道闭锁患儿被成功筛查的标准。统计上海地区胆道闭锁发病情况,并通过计算本次筛查敏感度、特异性、手术日龄及早期手术率,分析其对胆道闭锁早期诊断及治疗的影响。结果上海市四家胆道闭锁诊治中心数据汇总显示,本次研究期间上海地区总共出生41例胆道闭锁患儿,发病率为10.86/10万活婴(41/377 420,95%CI:7.80～17.74)。共有95例疑似患儿被筛查中心识别,11例患儿被确诊为胆道闭锁,其中9例在42 d回访前自行联系本中心;...     

The evaluation of infant stool color card combined with laparoscopy-assisted cholangiography in diagnosis of prolonged jaundice in infants

目的 探讨大便比色卡联合腹腔镜胆管造影在婴儿延迟性黄疸诊断中的价值.方法 72例延迟性黄疸患儿(男34例,女38例,平均日龄64 d),由父母连续7d以上进行大便颜色观察并与比色卡对照:大便颜色持续异常者,以及大便颜色波动在正常与异常之间经内科治疗无效的患儿直接行腹腔镜探查和胆管造影、肝脏组织病理检查;大便颜色持续正常者,以及大便颜色波动在正常与异常之间经内科治疗转为正常的患儿则行内科治疗.所有患儿均行B超、MRCP、ECT检查并随访6～12个月.结果 48例患儿大便颜色连续7d以上为异常,腹腔镜探查及胆管造影结果示:43例为胆道闭锁(BA),5例为非BA.14例患儿大便颜色持续至少7d正常,诊断为婴肝综合征(IHS),内科治疗并随访6～12个月全部治愈.10例患儿大便颜色波动在正常和异常之间,4例经内科治疗后大便颜色转为正常,排除BA行内科治疗,随访3例治愈,1例为漏诊BA;6例内科治疗后无变化,腹腔镜检查诊断1例为IHS,3例为BA,2例为胆道发育不良(CBDH).72例患儿中8例(11.1％)非BA接受了腹腔镜胆管造影,大便比色卡诊断BA的灵敏度、特异度和准确率分别为97.9％、73.9％和90.0％.结论 大便比色卡筛查延迟性黄疸患儿,有选择地行腹腔镜检查,显著降低了IHS患儿行腹腔镜检查比率,提高了BA的早期诊断准确性.     

比色卡提高医生对陶土色大便识别的调查研究

目的了解大便比色卡能否提高医生对陶土色大便的识别率及医生对胆道闭锁的认知情况。方法采用自行设计的电子问卷对儿童保健科医生进行问卷调查。问卷中列出12张大便照片,包括5张正常婴儿大便和7张胆道闭锁患儿的陶土色大便,提供大便比色卡比对前后,请医生辨认颜色正常大便或不正常大便,同时调查胆道闭锁相关知识。结果共收集问卷200份,参与问卷调查者来自国内24个省市的医疗单位,90名(45. 0%)医生首次能正确识别出所有陶土色大便。仅有113名(56. 5%)医生接诊黄疸患儿时关注大便颜色,87名(43. 5%)医生接诊黄疸患儿较少或不关注大便颜色,96名(48. 0%)医生对胆道闭锁这个疾病不熟悉,50名(25. 0%)医生错误地认为胆道闭锁以间接胆红素升高为主,104名(52. 0%)医生未听说过大便比色卡。在提供大便比色卡比对后,儿童保健科医生识别陶土色大便照片为异常大便的总体比例从81. 9%提高到93. 8%,并且对于图1中的2号、3号、6号、9号、11号陶土色大便照片正确识别率均有明显提高(P 0. 05)。经过大便比色卡对比后,三级医院儿童保健科医生识别陶土色大便照片为异常大便的总体比例从83. 7%提高到93. 1%(P 0. 05);一、二级医院儿童保健科医生识别陶土色大便照片为异常大便的总体比例从80. 3%提高到94. 4%(P 0. 05)。按医生职称分为主治医师及以下组和副主任医师及以上组,经过大便比色卡对比后,两组医生对陶土色大便正确识别率明显升高(P 0. 05)。结论儿童保健科医生对胆道闭锁认识和警惕性不足,大便比色卡能提高医生对陶土色大便的正确识别率,推广大便比色卡的应用及加强对于胆道闭锁基础知识的宣传及学习非常必要。     

产前诊断胆道闭锁影像学研究

目的：胆道闭锁早期诊断困难,而产前诊断更是极少发现。本文对产前诊断的胆道闭锁影像学特点进行探讨。方法回顾我院2010年至2012年收治的产前诊断9例产前诊断胆道畸形患儿,入院手术年龄24 d至2岁。全部患儿行腹腔镜胆道造影,4例诊断胆道闭锁,5例诊断先天性胆管扩张症,胆道闭锁患儿中2例接受腹腔镜下肝管空肠ROUX-Y吻合术,2例接受开腹肝门空肠吻合术。观察其临床表现,超声和实验室指标,术中情况,术后恢复情况等。结果4例患儿产前超声未见胆囊或胆囊显示不清。产前超声发现肝门部囊肿的3例患儿,囊肿小且均无明显增大,张力较高,呈规则圆形。产前诊断发现肝门囊肿的3例患儿术中证实为胆总管远端闭锁（Ⅰ型胆道闭锁）,未发现胆囊也未发现囊肿的1例患儿证实为Ⅲ型胆道闭锁。生后全部胆道闭锁患儿出现黄疸,最早出现在生后第2天,但都未出现陶土样便。全部胆道闭锁患儿囊肿大小形态无明显变化。全部患儿查ALT、AST、rGGT、直接胆红素和总胆红素进行性升高。2例接受腹腔镜下肝管空肠ROUX-Y吻合术,2例接受开腹肝门空肠吻合术。全部4例患儿术后恢复好。结论产前超声检查可以确定胆道闭锁,如果发现肝门部囊肿的胎儿,应定期接受超声检查,如果囊肿在孕期变化不明显,应怀疑囊肿型胆道闭锁。如产前超声未发现胆囊结构,则应怀疑为Ⅲ型胆道闭锁。生后应密切观察、超声、生化、黄疸情况。如果黄疸进行性加重可及早进行腹腔镜胆道造影及手术治疗。     

ECT对诊断新生儿胆道闭锁的临床意义

先天性胆道闭锁胆道闭锁是新生儿期一种严重黄疸疾病 ,它与新生儿肝炎鉴别很重要 ,前者必须手术治疗 ,而后者的治疗以保肝治疗为主。胆道闭锁早期的临床症、实验室检查、肝脏病理改变等方面 ,与新生儿肝炎颇相似 ,故两者的早期鉴别诊断十分困难。我们应用单光子发射型计算机断层照机机 ( Singlephoton emission computed tomography,SPECT,ECT)辅助诊断 2例胆道闭锁 ,报道如下。病例报告例 1男 ,2月 ,因皮肤黄染 2个月而入院。生后 3天起全身皮肤出现黄染 ,以后持续不退 ,且黄疸逐渐增深 ,大便呈灰白色 ,尿色黄。经强的松、苯巴比妥及保…     

胆道闭锁围生期危险因素的病例对照研究

摘要 目的：探讨胆道闭锁的危险因素,为早期鉴别诊断及其病因和发病机制的研究提供可能的线索。方法：自行设计胆道闭锁危险因素调查问卷,包括患儿自身、母亲、父亲和环境共22项因素,对2016年7月21日至2017年6月21日在重庆医科大学附属儿童医院肝胆外科因不明原因黄疸住院的＜7月龄患儿在入院时行问卷调查。在每个病例随访至少3个月后并从病志中截取所有调查对象的临床资料,确定胆道闭锁组和对照组,并按性别、年龄±10 d行两两匹配。先行单因素分析后,以结果中P<0.2的指标为自变量行多因素Logistic回归分析,考察问卷中的各项因素与胆道闭锁的相关性。结果：发放调查问卷146份,均回收,满足胆道闭锁组、对照组的纳入和排除标准者分别为96和42例,胆道闭锁组与对照组两两匹配后各42例进入本文分析。单因素分析显示,孕期四维彩超胆道异常、低出生体重、出生时不良事件、生后大便颜色变浅、母亲孕期补充微量元素、孕期补充叶酸、孕期存在并发症在两组间差异有统计学意义。多因素Logistic回归分析显示,母亲存在孕期并发症（OR=21.27,95%CI：3.50~129.21）、患儿生后大便颜色变浅（OR=6.70,95%CI：1.73~26.02）是胆道闭锁的独立危险因素;低出生体重是胆道闭锁发病的独立保护因素（OR=0.05,95%CI：0.00~0.67）。结论：母亲存在孕期并发症（妊娠期糖尿病、高血压、肝内胆汁淤积症）、生后大便颜色变浅可能是胆道闭锁的独立危险因素,对于胆道闭锁与其他黄疸性疾病的早期鉴别诊断有一定意义。     

腹腔镜在诊断先天性梗阻性黄疸中的初步应用

梗阻性黄疸在新生儿期甚为多见 ,部分病例早期诊断困难 ,本组 5例经腹部Ｂ超、ＣＴ、ＭＲＣＰ等检查无法鉴别是先天性胆道闭锁还是新生儿肝炎 ,经腹腔镜检查 ,3例确诊为新生儿肝炎 ,2例为新生儿胆道闭锁 ,并得到相应及时治疗。一、资料和方法1.年龄与性别　 1999年 1月～1999年 12月同济医科大学附属协和医院小儿外科和武汉市儿童医院共收治5例阻塞性黄疸患儿 ,均为男性 ,年龄 40～ 80ｄ ,平均 6 5ｄ。2 .临床表现　患儿均在生后 1周左右出现黄疸 ,且进行性加重 ,伴陶土色大便 ,深茶色小便。体检 :腹壁静脉显露 ,肝脏肿大 ,肝下界于右锁骨中…     

胆道闭锁15例

目的探讨胆道闭锁(BA)的临床特点、治疗与预后的关系。方法回顾性分析经中国医科大学附属盛京医院小儿外科近2 a来诊治的15例BA患儿的临床症状、体征、实验室检查结果,诊断、治疗经过及预后,并结合文献报道,对其发病原因、治疗经验、疾病转归及预后进行综合分析。结果男9例,女6例;年龄28~90 d。胆总管闭锁型(Ⅰ型)、肝管闭锁型(Ⅱ型)各3例,肝门区胆管闭锁型(Ⅲ型)8例,胆总管闭锁并先天性肠旋转不良1例。11例生后即出现黄疸,4例出生1周内逐渐发生黄疸。大便进行性变浅或表现为陶土样便13例,2例生后2 d即排陶土样便。15例胆红素水平均有升高,且排泄性肝胆超声均表现为胆囊收缩不良。并巨细胞病毒感染14例,感染率为93%。患儿均行手术治疗,术后病理结果均表现为胆汁淤积性肝硬化。10例患儿术后6~28 d出院,5例患儿术后2 d家属放弃治疗出院。目前随访患儿共12例,随访时间1个月~1.5 a,生长发育均良好,9例有黄疸,10例有黄绿色便排出,2例排陶土样便,4例Ⅰ型和3例Ⅱ型的BA患儿胆红素水平术后可降至正常水平,2例Ⅲ型BA患儿胆红素在术后1个月左右降至正常水平,但反复发生毛细胆管炎。结论BA的病因与病毒感染...     

婴儿巨细胞病毒感染与胆道闭锁的关系

目的　探讨巨细胞病毒 (CMV)感染与胆道闭锁的关系 ,了解婴儿CMV肝炎并胆道闭锁的临床特点。方法　对确诊为CMV感染并胆道闭锁 1 6例患儿的临床资料进行回顾性分析 ,并与同期诊断为单纯CMV肝炎 2 9例患儿进行比较。结果　CMV感染并胆道闭锁患儿血清CMV IgM抗体和外周血多形核白细胞中CMV pp65抗原均阳性 9例 ,IgM阳性 1例 ,仅pp65阳性3例 ,IgM和pp65均阴性 3例 (但其肝组织CMV pp65阳性 )。 1 5例肝组织标本中CMV pp65阳性 1 4例。CMV感染并胆道闭锁患儿各项指标明显重于有黄疸的单纯CMV肝炎 (P均 <0 .0 5) ,肝组织病理检查 1 5例显示胆小管增生伴肝纤维化 ,继发性胆汁性肝硬化 2例。结论　CMV感染可同时累及肝细胞和胆管上皮细胞 ,导致胆管闭锁。对以胆汁淤积为主要表现且已明确为CMV感染患儿应警惕是否并胆道闭锁 ,避免丧失手术治疗机会。     

胆道闭锁疑似病例的临床特征和诊断依据分析

目的 回顾手术确诊非胆道闭锁和胆道闭锁两组病例的临床资料,分析胆道闭锁疑似病例的临床特征和术前疑诊为胆道闭锁的原因.方法 收集2004年至2010年本院术前拟诊为胆道闭锁而行腹腔镜或开腹胆道造影的602例患儿的临床资料,依据术中胆道造影的诊断结果分为非胆道闭锁组和胆道闭锁组.分析近年非胆道闭锁病例所占比例,对照两组患儿黄疸发生日龄、肝功能及B型超声结果.计算同位素肝胆排泄性造影诊断胆道闭锁的阳性预测值及假阳性率,以及非胆道闭锁病例的疾病构成.结果 非胆道闭锁组83例,胆道闭锁组519例.近年行手术探查的所有患儿中非胆道闭锁比例无明显下降.两组出现黄疸的日龄、入院TBIL(169.9 mmol/L比172.3 mmol/L,P＞0.05)、DBIL(128.7 mmol/L比132.5 mmol/L,P＞0.05)、DBIL/TBIL(0.76比0.77,P＞0.05)、ALT(141.3 mmol/L比114.9 mmol/L,P＞0.05)比较均无统计学差异.γ-GT非胆道闭锁组显著低于胆道闭锁组(263.2 mmol/L比902.7 mmol/L,P＜0.01),B型超声检查肝脏肋下大小,非胆道闭锁组小于胆道闭锁组(2.99比3.61,P＜0.01).同位素肝胆排泄性造影阳性患儿共498例,其中术后诊断为非胆道闭锁患儿66例,假阳性率为13.3％.83例非胆道闭锁组包括:婴儿肝炎综合征58例,胆道发育不良16例,TPN相关性胆汁淤积症5例,胆道穿孔和浓缩胆栓综合征各2例.结论 肝功能检查的相似性及过度依赖同位素肝胆排泄性造影可能是非胆道闭锁患儿疑诊为胆道闭锁的主要原因,术前仔细分析黄疸出现的日龄、γ-GT、B型超声检查肝脏大小有利于非胆道闭锁病例术前的鉴别诊断.     

关节内侧间隙测量对髋关节脱位术后稳定性的预测

目的探讨关节内侧间隙测量对髋关节脱位术后稳定性的预测意义。方法分析2004～2007年本院10例髋关节再脱位患儿以及随机抽取的50例术后未发生再脱位患儿的影像学资料,对其术后1d、1周、1．5个月、3个月、4个月、5个月、6个月骨盆平片进行患侧髋关节内侧间隙值OA以及泪滴至髋臼外缘的距离OA的测量,并采用D值（D=OA／OA）进行标准化处理。结果无再脱位组D值基本小于0．8。其中30例采用髋关节外展支具,未出现再脱位,D值位于0．66～0．8之间;脱位组在石膏同定期间,D值基本小于0．7,此时股骨头位于髋臼内,未出现脱位;术后6周至3个月拆除石膏后,D值为0．66．0．8,未采取措施,相继出现脱位,此时D值基本大于0．8。结论测量关节内侧间隙对于评价髋关节脱位术后关节的稳定性以及预测早期再脱位有重要意义。D值小于0．66,关节稳定,不会出现再脱位;D值为0．66～0．8,关节稳定性受到影响,可能出现再脱位,需尽早采取干预措施;D值大于0．8,出现再脱位,简单保守治疗措旆失去作用．需再次手术蚕新复位。     

Tacrolimus: the good, the bad, and the ugly

The aim of this study was to evaluate the efficacy and side-effects of tacrolimus in pediatric transplant patients previously receiving cyclosporin A (CsA). This study was a retrospective chart review strengthened by a concomitant patient interview. Eleven pediatric cardiac or renal transplant patients, who had been converted from CsA to tacrolimus from October 1995 to January 1999 at The Cleveland Clinic Foundation, were included; there were six renal and five cardiac transplant patients. Each chart was reviewed to assess transplanted organ function pre- and post-conversion. For the six renal transplant patients, creatinine levels and biopsy findings were evaluated. For the five cardiac transplant patients, cardiac catheterization and routine biopsy data were analyzed likewise. Epstein Barr virus (EBV) status was also evaluated in each patient. In addition, each parent or patient was interviewed to ascertain dates of transplant, current medications, and side-effects. The patients' ages ranged from 6 to 20 yr (mean age 14.6 yr). All patients had been converted to tacrolimus. Eight patients were converted for treatment of refractory rejection, two were converted because of CsA-associated side-effects, and one patient was converted empirically for a history of multiple previous transplant rejections. Seven out of eight patients who received tacrolimus for rejection therapy improved. One patient had complete resolution of gingival hyperplasia. Another patient who previously developed hemolytic uremic syndrome on CsA had no further evidence of hemolysis. Four patients were weaned off steroid therapy. Despite conversion, two renal transplant patients progressed to chronic rejection. Five patients exhibited no side-effects. Side-effects experienced included transient hyperglycemia in conjunction with steroid use, headaches, and tremors that subsided rapidly. Four of 11 patients developed post-transplant lymphoproliferative disease (PTLD). Fortunately, reducing the dose of tacrolimus and/or surgical resection of the mass (if present), eradicated the disease. In conclusion, conversion therapy successfully provides an alternate treatment for acute rejection. It also enabled some patients to discontinue steroid therapy, maximizing growth potential. PTLD is a severe, potentially life-threatening complication that needs to be recognized and monitored closely. In conclusion, tacrolimus has been shown to be a very effective agent for the treatment of refractory organ rejection, but must be used cautiously.     

Neuroimaging in the developmental disorders: the state of the science

The developmental disorders of childhood autistic, developmental language, reading (dyslexia), and attention deficit-hyperactivity disorders-manifest with deficits in the traditional behavioral domains of cognition, language, visual-spatial function, attention, and socialization. However, none of these disorders has been associated with characteristic discrete focal lesions or recognized encephaloclastic processes. Developmental cognitive neuroscientists must therefore begin with the spectrum of sometimes divergent behaviors occurring within these disorders and work backward in an attempt to identify the responsible anomalous neural systems. Since the advent of "brain imaging" two decades ago, much effort has focused on identifying brain-behavior correlates in these disorders. The results of these neuropathologic, structural, and functional neuroimaging studies are presented and the reasons for the often divergent findings are discussed. As we approach the end of the Decade of the Brain, current neuroimaging techniques give us the technology for the first time to apply a fundamental cognitive approach to brain-behavior relationships in the developmental disorders, to eliminate the conglomeration of "apples and camels" phenomenon. Researchers are working together to create comparable protocols and to adhere to methods that can be replicated across sites. The future prospects for a greater understanding of the developmental disorders are now much brighter with neuroimaging technology.     

Flaws in the assessment of the best interests of the newborn

We examined the literature on ethical decisions regarding neonates, to assess whether personal beliefs and prejudices influence end-of-life decisions taken by caregivers. Studies show that religion and familiarity with disability influence caregivers' decisions, whereas the influx of already being a parent, age, sex and professional experience is controverse. Caregivers' attitudes towards end-of-life decisions are also affected by personal concerns about litigation, prejudices and their view of disability. The concept of 'poor quality of life' is widely used as a reference in end-of-life decisions, but this can be interpreted differently, leaving room for a wide range of personal viewpoints. In most cases, parents' opinions are considered important and are sometimes the main determinant in decision making. However, it is unclear whether parents' decisions are based on their own wishes or on the best interests of the newborn.
Conclusion: In neonatal end-of-life decisions, patients may not receive cures based only on their best interests.     

抗癫(癎)药物对骨代谢的影响和防治研究进展

癫痫是神经科常见病,目前控制癫痫发作的主要手段是药物治疗。抗癫痫药物（anti—epileptic drugs,AEDs）对骨骼代谢的影响在上个世纪60年代末就有报道,近年来广大学者对此进行了深入的研究。现就国外关于AEDs对骨代谢的影响、监测和防治的研究作综述如下。     

Effect of the adaptation of the food of the hospitalized child on the cover of the nutritional needs]

AIMS: Denutrition remains a major concern in hospitalized children. Daily experience suggests that the meals proposed by hospital dietetic service, although well-balanced and in accordance with the recommendations, may be poorly accepted and consumed by children. The aims of this study were to assess the effect of modification of foods offer on energy intakes as well as nutriments and minerals and trace elements in hospitalized children. PATIENTS AND METHODS: During a 1-month period, 25 consecutive children (range 4-17 years; 13 girls), hospitalized in our pediatric department were included in the study (reasons for hospitalisation comprised: medical reasons [n=7], orthopedic problem [n=16] or surgery [n=2]). They had no restricted diet and received the usual pediatric hospital feeding according to the French recommended dietary allowances (RDA) (D1). They were compared to 21 children--matched for age, sex, nutritional status and pathology, hospitalized during the following 1-month period--who received a modified diet (D2), elaborated by dieticians according to the child's preference and excluded or limited food usually nonconsumed by the children. Food consumption was prospectively measured for 24h by analysis of the nonconsumed foods, as well as browsing and extra food brought by the family. Analysis of energy, carbohydrate, lipid, protein, iron and calcium intake was made using Bilnut 3 software (Nutrisoft, France). RESULTS: D2 covered 119+/-37% of the median energy needs versus 89+/-37% for D1 (p<0.05). The median energy needs were more often reached with D2 as compared to D1 (62% versus 32%, p<0.05). Protein intake was high in both groups, more importantly with D2 (266+/-111% of RDA versus 193+/-77% with D1, p<0.05). We observed no difference between the 2 diets in regards of fat/carbohydrate balance and iron intake. Calcium intake was increased with the adapted diet: 68+/-26% of RDA with D2 versus 49+/-26% with D1 (p<0.01). CONCLUSION: Adapting food offers to preference influences food and caloric intakes in hospitalized children. This could be an efficient strategy to prevent acute undernutrition in hospital.