Bile acid metabolism in extrahepatic biliary atresia: lithocholic acid in stored dried blood collected at neonatal screening

Lithocholic acid (LCA) is a potent hepatotoxic compound. Fetal LCA may have a role in the pathogenesis of neonatal cholestasis/extrahepatic biliary atresia (EHBA). Fetal liver efficiently hydroxylates LCA in several positions. This may represent a detox-ification mechanism. In the present study LCA, cholic acid (CA) and chenodeoxycholic acid (CDCA) were quantitated by gas chromatography-mass spectrometry using selected ion monitoring in small amounts of stored dried blood from six newborn infants with EHBA and fourteen con-trols. The blood was collected at neonatal metabolic screening. Mean blood levels (+/- S.E.M.) of LCA were 0.11 +/- 0.04 microM in the in-fants with EHBA and 0.08 +/- 0.02 microM in the control infants. The correspon-ding levels for CA and CDCA were 15.6 +/- 3.6 microM and 7.4 +/- 2.5 microM in the infants with EHBA and 1.7 +/- 0.3 microM and 1.8 +/- 0.4 microM in the controls. The increased levels of CA and CDCA in the infants with liver disease can be explained by cholestasis. The low blood levels of LCA indicate a normal fetal metabolism of this bile acid in EHBA.     

胆道闭锁和新生儿肝炎肝脏的电镜观察

对6例胆道闭锁患儿和3例新生儿肝炎患儿肝脏进行了透射电镜的观察。发现胆道闭锁患儿肝细胞质内高电子密度物质(EDM)较新生儿肝炎患儿为多。认为其与胆汁淤滞有密切关系。推测其为胆色素性物质。胆道闭锁患儿毛细胆管数量较新生儿肝炎患儿为多,管腔稍有扩张。还可见到胆道闭锁患儿毛细胆管处细胞连接破坏现象。这些可能是胆管梗阻,胆管内压增高所致。     

实体肿瘤患儿血清AFP监测

目的：了解监测血清甲胎球蛋白（AFP）诊断小儿恶性实体肿瘤的意义。方法：采用放射免疫分析法，对106例小儿实体肿瘤、24例新生儿黄疸（包括17例新生儿肝炎和7例胆道闭锁）和30名正常儿童血清AFP进行测定。85例（80％）肿瘤患者术后随访1个月－7a。结果：78.6%肝母细胞瘤、88.9%卵黄囊瘤、31.6%骶尾部畸胎瘤、10％肾母细胞瘤和所有新生儿黄疸患儿AFP测定值同正常儿童相比明显升高（P＜0.02）。肿瘤切除后AFP多在术后2－6周恢复正常。AFP假阳性结果多发生在新生儿或小年龄组的婴幼儿。结论：测定AFP可作为诊断小儿肝母细胞瘤和卵黄囊瘤及判断肿瘤是否被完整切除和手术后有无复发的重要依据之一，但在新生儿和婴幼应注意假阳性结果。     

~(131)碘玫瑰红肝胆连续扫描对婴儿黄疸的诊断

本文应用~(131)碘-玫瑰红对19例黄疸婴儿作了检查。结果显示:13例胆道闭锁患儿在注射~(131)碘-玫瑰红后24小时仍无肠影出现;5例新生儿肝炎肠道显影;另1例胆道狭窄肠道显影延迟(假阴性)。用本法连续扫描检查方法简便、安全、准确性较高。诊断的关键在于熟悉各种类型黄疸图像的特点以及细致的分析。     

小儿胆道闭锁症诊断与处理的改进

回顾了4年来18例胆道闭锁症的诊断与处理。指出以十二指肠引流液胆红素及胆酸测定鉴别胆道闭锁症与新生儿肝炎简便易行,可以缩短术前诊断时间。术中使用稀释美兰胆道造影代替X线胆道造影也可以了解肝外胆道的形态。作葛西手术时适当切断肝脏冠状和镰状韧带托出肝脏,在腹腔外解剖肝门可以降低手术的难度,便于完成肝门肠吻合操作。     

Screening for biliary atresia

Biliary atresia is a severe progressive cholangiopathy which leads to early liver cirrhosis and is uniformly fatal. Early surgical intervention (the Kasai procedure) is needed for an improved outcome. However, early recognition and diagnosis is not easy during the neonatal period because of the high incidence of neonatal jaundice, ill-informed and less than urgent appraisal of the clinical manifestations among jaundiced neonates. A mass screening program for biliary atresia using a stool color card was conducted in Taiwan from March 2002 (in 49 hospitals and clinics) to December 2003 (in 95 hospitals and clinics). The stool color card for infants has proved to be a sensitive and specific screening method for biliary atresia in infants younger than two months of age.     

胆道无扩张的新生儿和婴儿梗阻性黄疸的治疗

研究56例无胆道扩张的新生儿及婴儿梗阻性黄疸的临床特点及外科手术时机及效果。超声证实无肝内外胆道扩张的新生儿及婴儿梗阻性黄疸,均行手术探查及病理学检查。手术证实56例梗阻性黄疸中有30例为胆汁粘稠症,并经胆道冲洗治愈;26例为胆道闭锁,预后不良。二者在肝内小胆管增生这一病理改变上存在一致性,可能是对胆道梗阻的代偿机制之一。对于超声提示无肝内外胆道扩张的新生儿及婴儿梗阻性黄疸,经1～2周的抗炎、保肝、利胆治疗无效后,应及时手术探查,以使胆汁粘稠症的患儿得到有效治疗。     

110例胆道闭锁患儿临床资料分析

目的回顾性分析110例胆道闭锁患儿的临床特征以为胆道闭锁的诊断和治疗提供更多证据。方法收集2017年8月至2020年8月期间在深圳市儿童医院收治的110例胆道闭锁患儿的临床资料,对患儿的一般资料和实验室检查资料进行总结分析。结果本研究纳入的110例胆道闭锁患儿中,男49例(39%),女67例(61%)。在所有患儿中仅有4例为早产儿;87例(79%)患儿出现白陶土样便;30例(28%)患儿的γ-谷氨酰转肽酶(γ-GT)<300 u/L,79例(72%)患儿的γ-GT≥300 u/L。比较γ-GT<300 u/L组和γ-GT≥300 u/L组两组患儿的临床特征,发现γ-GT<300 u/L组的ALT和AST水平均显著高于γ-GT≥300 u/L组(P<0.05)。巨细胞病毒病原学检查发现13例患儿呈阳性。结论本研究发现胆道闭锁多见于足月儿,且伴有持续性黄疸及白陶土样大便,多数患儿的γ-GT高于300 u/L。     

Outcome of extremely low birthweight infants at the University Hospital of the West Indies, Jamaica

OBJECTIVE: To describe the early outcome of extremely low birthweight infants delivered at the University Hospital of the West Indies. METHODS: A two-year retrospective review of the charts of all live, inborn extremely low birthweight infants admitted to the neonatal unit between January 1, 2002 and December 31, 2003 was conducted Differences between survivors and non-survivors were determined using analysis of variance and predictors of outcome were determined using multiple regression models. RESULTS: During the study period, 47 extremely low birthweight infants were admitted to the neonatal unit. The mean +/- SD birthweight and gestational age of these infants were 780 +/- 137 g and 27 +/- 2 weeks respectively. Twenty (43%) infants survived Babies (19; 58%) of gestational age > or = 27 weeks had increased survival compared to those < 27 weeks, (1; 7%; p = 0.001) and babies weighing > or = 750 g had increased survival (17, 65%) compared to those weighing < 750 g, (3, 14%; p < 0.001). Infants delivered by Caesarean section had improved survival 15 (58%) over those delivered vaginally (5, 24%; p = 0.02). All six (100%) infants whose mothers did not receive prenatal steroids died while 18 (50%) infants whose mothers received prenatal steroids died (p = 0.02). Significant factors associated with outcome were offered and gender was entered into a multiple regression model; gestational age and female gender remained independent predictors of survival. CONCLUSION: Obstetric measures for the prevention of preterm delivery need to be optimized in order to decrease the morbidity and mortality associated with extremely low birthweight infants.     

Biliary atresia associated with meconium peritonitis

Biliary atresia, malrotation, meconium peritonitis and transient hypothyroidism are occasionally seen in neonatal infants. Biliary atresia associated with malrotation has been reported in some patients with polysplenia syndrome, but biliary atresia associated with meconium peritonitis has only been described by a few investigators. Here we present a case of meconium peritonitis due to malrotation with volvulus, followed by biliary atresia and transient hypothyroidism during early infancy.     

Biliary atresia--translational research on key molecular processes regulating biliary injury and obstruction

Biliary atresia is the most common cause of pathologic jaundice in young infants and results from the obstruction of the extrahepatic bile ducts by an inflammatory and fibro-obliterative process. Although the pathogenesis of the disease is multifactorial, recent patient- and animal-based studies began deciphering the molecular pathways involved in biliary injury and duct obstruction. Using large-scale genomics and immunostaining of livers from children with biliary atresia, investigators have discovered unique molecular signatures of dominant proinflammatory cytokines at the time of diagnosis. To study hypotheses generated from these patient-based studies, the anatomical and inflammatory profiles of a mouse model of rotavirus-induced biliary atresia were analyzed and found to share striking similarities with the human profiles. Then, using these mice in mechanistic studies, interferon-gamma (IFNgamma) has been shown to regulate the biliary tropism of lymphocytes to the biliary system, and to play a critical role in the inflammatory obstruction of extrahepatic bile ducts. The ability to combine human studies with a laboratory model of neonatal biliary injury and obstruction opens a new era of opportunities to advance the field of biliary atresia, and to develop new therapeutic strategies to improve long-term outcome with the native liver of children with biliary atresia.     

Bile Acid Metabolism in Extrahepatic Biliary Atresia

Lithocholic acid (LCA) is a potent hepatotoxic compound. Fetal LCA may have a role in the pathogenesis of neonatal cholestasis/extrahepatic biliary atresia (EHBA). Fetal liver efficiently hydroxylates LCA in several positions. This may represent a detox-ification mechanism. In the present study LCA, cholic acid (CA) and chenodeoxycholic acid (CDCA) were quantitated by gas chromatography-mass spectrometry using selected ion monitoring in small amounts of stored dried blood from six newborn infants with EHBA and fourteen con-trols. The blood was collected at neonatal metabolic screening. Mean blood levels (±S.E.M.) of LCA were 0.11±0.04 μM in the in-fants with EHBA and 0.08±0.02 μM in the control infants. The corresponding levels for CA and CDCA were 15.6±3.6 μM and 7.4±2.5 μM in the infants with EHBA and 1.7±0.3 μM and 1.8±0.4 μM in the controls. The increased levels of CA and CDCA in the infants with liver disease can be explained by cholestasis. The low blood levels of LCA indicate a normal fetal metabolism of this bile acid in EHBA.     

母体乙肝病毒感染与新生儿黄疸关系的临床研究

目的探讨母体乙肝病毒感染与不同类型新生儿黄疸的关系。方法385例病理性黄疸新生儿患者根据母体乙型肝炎抗原阳性与否分为肝炎组与非肝炎组，比较两组患儿溶血性黄疸与G-6PD缺乏症黄疸比例，并比较不同黄疸类型情况下，两组患儿胆红素水平差异。结果（1）母体乙型肝炎抗原阳性者52例，乙型肝炎抗原阴性者333例。肝炎组与非肝炎组乙型新生儿溶血病，G6PD缺乏症以及其他原因导致溶血发生率分布情况差异有统计学意义（Y0：385．000，P〈0．001）。乙型肝炎抗原阳性组G6PD缺乏症阳性率为34．6％，乙型肝炎抗原阴性组G6PD缺乏症阳性率为22．5％。两组患儿新生儿溶血病阳性率差异相对比较小，分别为50．5％与42．3％。（2）G6PD缺乏症患儿中，母体乙型肝炎抗原阳性组最高总胆红素水平与乙型肝炎抗原阴性组差异有统计学意义（t=4．672，P〈0、01）。非G6PD缺乏症溶血，如新生儿溶血病与其他类型黄疸，母体乙型肝炎抗原阳性患儿组最高总胆红素水平与乙型肝炎抗原阴性组无显著差异（P〉0．05）。结论新生儿黄疸中，母体乙型肝炎抗原阳性者新生儿G-6PD缺乏症发生率高，并与G-6PD缺乏症病情有关。     

Neonatal jaundice: the surgical viewpoint.

There is good evidence that neonatal hepatitis, biliary hypoplasia, biliary atresia and choledochal cyst are different stages of one disease process for which the term infantile obstructive cholangiopathy has been suggested. Thanks to the work of Kasai and the operation of hepatic portoenterostomy the surgical outlook has greatly improved, although in North America it still leaves much to be desired. One cannot procrastinate too long in the hope that the patient's condition will improve spontaneously, because the surgical results are much better when the operation is performed before the patient is 10 weeks old. This article outlines the steps that should be followed in investigating neonatal jaundice, the nonsurgical measures that can be taken in an attempt to reverse or alleviate the underlying condition, and the specific role of the pediatric surgeon in the management of choledochal cyst and biliary atresia.     

单吻合器联合外翻脱出技术在直肠癌超低位前切除中的应用

目的探讨单吻合器联合外翻脱出技术在早期低位直肠癌保肛手术中的应用及其适应证和操作要点。方法回顾性分析早期低位直肠癌前切除保肛手术43例,按手术技术分为两组,实验组应用外翻脱出式单吻合器技术,对照组采用传统的双吻合器吻合技术。比较两组患者的肿瘤下缘与肛侧切缘的距离、局部复发率、并发症发生率、肛门控便功能以及手术费用等。结果远切缘长度:实验组为2.2 cm±0.2 cm,明显大于对照组1.9 cm±0.4 cm(P=0.006)。齿状线与远切线的距离:实验组1.3 cm±0.3 cm,对照组1.9 cm±0.5 cm,后者明显大于前者(P<0.001)。实验组无局部复发而对照组有3名患者术后1年内局部复发。实验组吻合口瘘发生率、手术器械费用均低于对照组,两组肛门控便、控气功能无明显差异,实验组1例夜间肛门周围有少量粪便污染,清醒时控便控气功能均好。结论直肠外翻脱出式单吻合器技术用于超低位直肠癌保肛手术具有手术切除彻底、安全性高、局部复发率低、肛门控便功能好、手术费用低等优点,尤其适用于早期低位直肠癌。     

应用组织芯片技术筛查胆道闭锁的病毒感染研究

目的应用组织芯片技术研究病毒感染与小儿胆道闭锁的关系。方法利用组织芯片技术检测42例胆道闭锁和5例对照组患者肝脏组织中的巨细胞病毒（CMV）和EB病毒（EBV）感染情况。结果在42例胆道闭锁患者肝脏组织中,其中CMV阳性26例（61．9％）,EBV阳性3例（7．14％）,对照组5例中均未检测到以上病毒的存在。结论胆道闭锁与病毒感染之间存在相关性,其中以CMV感染关系最为密切,可能作为其发病机制中的一个促发因素,最终引起胆道闭锁;抗病毒治疗可能会改善CMV感染EHBA预后。     

心区交感神经阻滞对严重心力衰竭左心室舒张功能的影响

目的观察心区交感神经阻滞对严重心力衰竭患者左心室舒张功能的影响。方法随机选择有严重心力衰竭,左心室射血分数<35%的扩张型心肌病患者39例,分为心区交感神经阻滞组(TEB组)和对照组。对照组给予常规抗心力衰竭治疗,TEB组在给予药物治疗的同时,予以TEB。于治疗前和治疗后1个月行超声心动图检查,测量左心房内径(Lad)、左心室内径(LVEDd)、左心室射血分数(EF),二尖瓣环6个位点心肌组织舒张早期速度和舒张晚期速度(Em和Am),并取平均值(MEm和MAm),比较两组上述指标变化。结果TEB组Lad、LVEDd显著缩小(LVEDd66mm±6mmvs71mm±6mm,P<0.05),EF显著增加(35%±7%vs23%±6%,P<0.05),二尖瓣环6个位点的Em和3个位点的Am均显著增加,MEm和MAm也显著增加(MEm5.7cm/s±1.5cm/svs7.1m/s±1.7cm/s,P<0.05),对照组Lad、LVEDd和EF无显著变化,二尖瓣环只有2个位点Em显著增加,Am、MEm和MAm均无变化。结论同常规治疗相比,TEB能在缩小严重心力衰竭患者左心室腔径、增强射血功能的同时显著改善左心室舒张功能。     

婴儿胆汁淤积症88例临床研究

目的探讨婴儿胆汁淤积症的临床特点和诊断方法。方法收集我科2009-2011年收治的88例胆汁淤积症患儿,对其病因、临床检查、病理及预后进行分析。结果婴儿胆汁淤积症男孩、足月儿多见;2~3月龄发病率高。本组患儿中胆道闭锁组19例;肝内淤胆组69例,其中仅10.1%明确病因。胆道闭锁组γ谷氨酰转肽酶(GGT)水平明显高于肝内淤胆组,其他生化指标差异无统计学意义。血筛瓜氨酸血症1例;尿筛40%患儿高度怀疑Citrin缺陷。肝内淤胆组中23例行LSC25A13基因12种突变位点的检测,结果均为阴性。胆道闭锁组B超确诊率为64.7%。49例肝组织病理提示两组胆汁性肝硬化发生率差异有统计学意义(P<0.01)。两组的病死率和好转痊愈率差异有统计学意义(P<0.01)。结论胆道造影术、内科动态观察是鉴别胆道闭锁的常用方法,前者更可靠。对于有肝内胆汁淤积病史或存在肝功能反复异常者,应积极找病因,注意代谢病检查,并长期随访。     

臀位分娩方式选择对新生儿影响的临床研究

目的：探讨不同臀位分娩方式对新生儿预后的影响。方法：回顾性分析自2007年1月至2011年12月期间在我院住院分娩的98例臀位产妇的临床资料,其中35例阴道分娩（臀位阴道分娩组）,63例剖宫产（臀位剖宫产组）,观察比较两组产妇新生儿死亡及新生儿窒息、吸入性肺炎等并发症发生情况,同时对两组新生儿进行Apgar评分并进45-比较。结果：63例臀位剖宫产组新生儿无1例死亡,35例臀位阴道分娩组新生儿3例（8．57％）死亡,两组新生儿死亡率差畀具有统计学意义（P〈0．05）。63例臀位剖宫产组新生儿,2例（3．18％）发生并发症,臀位阴道分娩组8例（22．86％）发生并发症,两组差异具有统计学意义（P〈0．05）。臀位剖宫产组新生儿Apgar评分为（7．2±1．8）分,臀位阴道分娩组为（4．5±1．3）分,前者显著高于后者,差异有统计学意义（P〈0．05）。结论：臀位分娩时选择剖宫产可降低新生儿并发症的发生,降低新生儿死亡率,提高Apgar评分,在臀位分娩时宜适当放宽剖宫产手术指征,对于改善新生儿预后有着重要意义．     

婴儿肝炎综合征与胆道闭锁鉴别诊断方法的比较

回顾性评价了经手术或尸检诊断的１１例胆道闭锁和随访了２６例黄疸消失的婴儿肝炎综合征患儿十二指肠液的颜色、放射性核素肝胆显像、Ｂ超肝胆检查结果．以十二指肠液透明无色诊断胆道闭锁，十二指肠液黄色诊断婴儿肝炎综合征，正确率为９４．６％．以肠道有无放射性示踪剂来鉴别胆道闭锁与婴儿肝炎综合征，正确率为８１．１％．Ｂ型超声波肝胆检查有无胆囊鉴别胆道闭锁与婴儿肝炎综合征，正确率为７８．０％．结果表明，观察十二指肠液颜色是简单、快速、可靠的鉴别方法．