T细胞亚群在新生儿胆管闭锁及肝炎综合征的早期诊断价值

目的：本课题通过探讨新生儿胆道闭锁、新生儿肝炎综合征在免疫方面的差异性,以寻找新的早期诊断有鉴别意义的实验室指标,为疾病的诊断、治疗（包括肝移植的机体的免疫状况）提供理论基础。方法：对2006年～2010年我院明确诊断为新生儿胆道闭锁、新生儿肝炎综合征患儿各40例,1～3月龄,观察两组治疗前机体的免疫状况。结果：BA组和NHS组CD_4、CD_8及CD_4/CD_8比值均较正常组下降,CD_4,CD_4/CD_8与正常组有统计学差异（P〈0.05）,且两者之间CD_4/CD_8有统计学差异（P〈0.05）。结论：新生儿肝炎和胆管闭锁的早期鉴别诊断一直是困扰临床医师的难题,T细胞免疫反应在新生儿胆道闭锁及新生儿肝炎综合征中扮演了重要角色,两者在免疫学方面的差异,特别是CD_4/CD_8比值很有可能成为临床快速、特异、简单、实用的鉴别诊断方法。     

超声检查对于新生儿黄疸的鉴别及诊断意义

目的探讨和研究超声检查对于新生儿黄疸的鉴别及诊断意义。方法对近两年来我院收治的44例黄疸患儿采用高频彩超进行检查,对胆囊形态和餐前、餐后变化情况进行记录和分析。结果本组44例患儿中包括42例肝炎综合征和2例胆道闭锁,肝炎综合征患儿中有36例餐前餐后的胆囊形态改变明显,而2例胆道闭锁患者则用餐后胆囊形态无变化,呈现出僵硬状态。结论高频超声检查新生儿黄疸的灵敏度较高,但特异性一般,尤其对于胆道闭锁的患儿而言敏感性较高,可以作为诊断此类疾病的有效方法进行推广和应用。     

胆道闭锁肝脏的超微结构研究

对９例先天性胆道闭锁患儿的肝脏进行了较系统的超微结构研究，指出肝细胞的变性坏死、毛细胆管扩张、肝细胞内胆汁淤滞和结缔组织增生是其超微病理特征，初步讨论了这些超微病理改变的机理，     

先天性胆道闭锁肝内毛细胆管超微结构与临床预后关系探讨

目的　探讨先天性胆道闭锁 (CBA)肝内毛细胆管超微结构与临床预后的关系。方法　用PHILIPSCM10透射电镜观察肝内毛细胆管超微结构 ,比较肝组织电镜切片中发育良好与发育不良的毛细胆管数目 ,并与临床预后作比较。结果　 2 5例CBA患儿中 ,肝内毛细胆管发育良好为主的 13例 ,其中 12例术后生存 ,生存率为 92 3% (12 / 13) ;毛细胆管发育不良为主的 12例中 ,仅有 4例生存 ,生存率为 33 3% (4 / 12 ) ,两组生存率有显著性差异 (P <0 0 5 )。结论　肝内毛细胆管的发育情况可作为初步判断术后临床预后指标之一。     

阻塞性新生儿肝炎胆道系统病变及其临床意义

目的观察分析阻塞性新生儿肝炎时其胆道系统的损害,以便指导临床诊疗工作。方法通过手术证实的22例阻塞性新生儿肝炎病儿,取肝组织活检、并在胆囊置管冲洗前后观察肝外胆管形态变化。结果22例阻塞性新生儿肝炎皆有不同程度的肝外胆道阻塞表现,管腔狭小,8例可见丝状胆栓影响排胆功能。结论阻塞性新生儿肝炎其与胆道受损是同步的。因此,临床上治疗肝炎的同时应做到输胆通畅,才有可能康复。     

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。     

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一一致性,可能是对胆道梗阻的代偿机制之一。     

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产闭锁，十二指肠黄色诊断婴儿肝炎综合征，正确率为９４．６％，以肠道有无效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     

其他结合胆红素增高症

新生儿结合胆红素增高症虽病因不同,但临床症状均以阻塞性黄疸为特征,即皮肤、巩膜黄染,大便也淡或呈灰白色,小便深黄,肝脾肿大及肝功能损害等,故以加以鉴别,进行病因治疗.1、分类1.1 肝胆道阻塞1.1.1 新生儿肝炎综合征1.1.2其他 ①胆道闭锁.②总胆管囊肿.③胆栓综合征.④总胆管结石.⑤自发性总胆管穿孔.③外源性胆管受压.     

胆管先天性疾病超声诊断

胆道闭锁 [病理与临床概要] 先天性胆道闭锁是胆道先天性发育障碍导致的胆道梗阻,是新生儿持续黄疸的常见原因,是小儿外科领域中最重要的消化外科疾病之一,也是小儿肝移植中最常见的适应征.其发生率在东南亚国家为1-9000,高于欧美国家的1:12-15000[1,2] .后者约有10%的患儿合并有其他器官先天性疾患如多脾症、器官转位、心血管缺陷和门静脉十二指肠前位等.至今明确的病因,有一些假说如:胚胎期发生异常、原始胆管重塑过程异常、病毒感染或其他的肝脏炎性反应.但上述的假说都无法获得普遍认同.可分为两种类型.①肝内型:较常见.肝内胆管完全闭锁,肝外胆道(包括胆囊)可全部闭锁或部分闭锁,也可以完全正常,此型手术难以矫正.     

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。     

~(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。     

Characteristics of Malaysian infants with biliary atresia and neonatal hepatitis

Cholestatic disorders of infancy (viz neonatal hepatitis and biliary atresia) have not been well studied in Malaysia. In a retrospective study in the Department of Paediatrics, University Hospital, Kuala Lumpur from January 1982 through December 1991, a total of ninety-three infants with such conditions were identified: 35 (38%) had biliary atresia, 58 (62%) neonatal hepatitis. There was a statistically significant male preponderance in the neonatal hepatitis group (P = 0.020). There was no significant difference in the racial distribution and in the proportions of low birthweight infants between the two groups of disorders. When the biliary atresia group was compared with the neonatal hepatitis group, significant differences were observed in the age of presentation (mean +/- SD) 9.8 +/- 6.8 VS 20 +/- 17.3 weeks (P < 0.001), proportion of infants with prolonged jaundice (> seven weeks) 28/35 (80%) VS 20/58 (34.5%) (P < 0.00001), occurrence of alcoholic stools 26/35 (74.3%) VS 27/58 (46.6%) (P = 0.020), liver size (mean +/- SD): 4.3 (1.6 cm VS 3.3 +/- 1.8 cm (P < 0.01) and splenic size: 2.5 (1.8 cm VS 1.4 (1.2 cm (P < 0.001). There was however considerable overlap between the two groups in these features at presentation, making clinical differentiation between the two conditions difficult. Infants with cholestasis tended to present late, compromising the chance of survival. In order to improve the medical care of these patients, these conditions must be emphasised during the training of medical practitioners, and efforts to increase public awareness of these conditions must be created.     

亚急性重症肝炎黄疸形成的肝脏组织学改变

本文通过组织学、组织化学及连续切片等方法研究了亚急性重症肝肝炎的肝脏组织学改变,认为胆管扭曲,假胆管是盲端等组织学改变是造成亚急性重症肝炎淤胆的一个因素。结合临床资料,把亚急性重症肝炎发病过程分为三个时期:1.血清转氨酶增高期,2.组织增生期,3.胆汁淤滞坏死期。提出胆汁性凝固性坏死这一概念并做描述。认为淤胆造成胆汁性凝固性坏死是亚急性重症肝炎发病过程的一个重要病理过程,对“胆酶分离”作了解释。     

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.     

先天性胆道闭锁的早期诊断研究进展

先天性胆道闭锁是新生儿胆汁淤积症最常见的病因,也是小儿肝移植的主要指征,如果不及时诊治,可导致患儿进行性肝纤维化、胆汁淤积性肝硬化甚至死亡。目前普遍认为及时行Kasai术或肝移植可明显改善先天性胆道闭锁患儿预后,因此其早期鉴别诊断极其重要。然而,先天性胆道闭锁与其他原因引起的新生儿胆汁淤积症早期在临床表现、实验室检查以及影像学特征方面存在着诸多共同点,给临床鉴别诊断带来极大困难。过去的十年里,各种无创性检查诊断先天性胆道闭锁取得了很大进展,包括早期筛查,特征性生物标志物的研发,超声检查定性、定量指标的完善,放射性核素肝胆动态显像价值所在的明确,经内镜逆行胰胆管造影术及磁共振胰胆管造影应用的探索等。但目前临床上尚无一种同时具有高敏感性和特异性的无创性早期鉴别诊断先天性胆道闭锁的方法。术中胆道探查、造影仍是目前诊断先天性胆道闭锁的最准确的方法,但该法的有创性及对术者的高要求限制了其在临床上的应用。本综述在阐述临床常用早期鉴别诊断先天性胆道闭锁方法特点的基础上,进一步探索了多学科、多技术联合应用早期鉴别诊断先天性胆道闭锁的最佳策略,为提高先天性胆道闭锁早期鉴别诊断的敏感性、特异性提供参考。      

Childhood liver diseases in Jos, Nigeria: a retrospective histopathological study.

Forty eight needle biopsies of the liver, from children registered in the histopathological laboratory of Jos University Teaching Hospital (JUTH) were reviewed. Liver disease diagnosis was based on histopathological criteria without recourse to either clinical, biochemical or microbiological data. Hepatic Schistosomiasis (37.5%) and liver cirrhosis (25%) were the most frequently diagnosed lesions. There were only two cases of biliary cirrhosis secondary to biliary atrisia. Parasitic infestation of the liver was the most common cause of childhood hepatic dysfunction. Our results confirm the observations of workers in other tropical and subtropical regions, where infection is the commonest cause of childhood liver disease. This is in sharp contrast to the findings from European countries where neonatal hepatitis or biliary atresia are the most commonly diagnosed disorders. This retrospective study provides a good starting point for a prospective study, to determine the incidence and severity of childhood liver disease in Nigeria. This is a retrospective histopathological study aimed at establishing the pattern of liver disease in the paediatric age group in Jos. The indication for liver biopsy in all the cases was hepatosplenomegaly with or without biochemical abnormality.     

婴儿肝炎综合征的病因分析及其肝组织的光镜和电镜的观察研究

本文报道了16例临床和病理诊断一致的婴儿肝炎综合征，其中14例用ELISA法检测多种病毒，并采用聚合酶链反应（PCR）法检测巨细胞病毒（CMV）－DNA，还用电镜观察了肝组织的超微结构变化。研究结果发现本组的主要病因是CMV感染，共有9例，占56．3％，其余为甲型肝炎病毒感染2例，EB病毒感染和肝外胆道闭锁各1例，还有3例原因不明。14例电镜下主要见肝细胞胞浆基质疏松，线粒体、内质网、高尔基氏器和糖原减少，溶酶体和脂滴增多。尽管本组病例的病因有多种，但在光镜和电镜下所见的病理变化基本一致。     

Managing liver failure

Liver disease is rare in childhood, but important new developments have altered the natural history and outcome. It is important that clinicians are aware of these diseases and their management. Acute liver failure is most often due to viral hepatitis, paracetamol overdose, or inherited metabolic liver disease. The clinical presentation includes jaundice, coagulopathy, and encephalopathy. Early diagnosis is necessary to prevent complications such as cerebral oedema, gastrointestinal bleeding, and renal failure. Early supportive management, in particular intravenous N-acetylcysteine, may be effective but liver transplantation is usually the definitive treatment and thus early referral to a specialist unit for liver transplantation is mandatory. Chronic liver failure may be due to unresolved neonatal liver disease, either inherited biliary hypoplasia or extrahepatic biliary atresia, while in older children, autoimmune liver disease or cystic fibrosis are the commonest causes. Treatment includes specific medication, nutritional support, and liver transplantation, which now has a 90% survival with good quality life.