胆道闭锁肝内外胆系组织病理形态学分析

目的 通过胆道闭锁（BA）肝门纤维块、肝脏组织的病理及其超微结构观察，对肝门成纤维细胞分化程度进行评分，并与肝纤维化分级进行相关分析。方法 选取BA患儿作为研究对象，术中取肝门纤维块及肝脏组织标本；研究同期选取疑似BA经术中胆道造影除外BA，诊断为胆汁淤积综合征和先天性胆管扩张症患儿作为对照组，留取肝脏组织标本。在光镜和电镜下观察标本的病理改变，以及肝细胞、毛细胆管和肝门成纤维细胞的超微结构。采用SPSS 14.0软件，半定量比较BA与对照组肝脏纤维化的差异，检验肝门纤维块成纤维细胞活跃程度与肝纤维化分级的相关性。结果 2005年7月至2006年5月复旦大学附属儿科医院收治的21例BA Kasai根治术病例，手术平均年龄（66±20）d；对照组为5例胆汁淤积综合征和10例先天性胆管扩张症患儿。BA组肝组织病理改变主要是肝内门脉区胆管炎症及纤维化形成，肝纤维化程度明显高于同年龄胆汁淤积综合征和先天性胆管扩张症患儿；肝门纤维块毛细胆管增生，部分管腔闭锁、狭窄，腔内炎细胞浸润及部分淤胆，大量间质成分增生；电镜下肝门成纤维细胞活跃、肝脏毛细胆管上皮微绒毛缺失、肝细胞及肝血窦内电子致密物质增多及部分毛细胆管扩张；肝门成纤维细胞分化程度与肝组织纤维化程度相关（P=0.04）。结论 BA肝组织病理改变主要是肝内门脉区胆管炎症及严重纤维化形成；超微结构改变提示肝门部成纤维细胞活跃，其分化程度与肝纤维化程度相关。     

胆道闭锁患儿血清sICAM-1与IL-18的变化及临床意义

目的　研究可溶性细胞间粘附分子 1(sICAM 1)、白细胞介素 18(IL 18)在胆道闭锁(BA)发病机制中的作用及临床意义。方法　胆道闭锁患儿 2 1例 ,男 8例 ,女 13例 ;年龄 2个月～ 12岁 ,所有患儿诊断均经手术后病理切片证实。正常对照 12例为健康体检儿童 ,年龄 1个月～ 10岁。采用ELISA法动态测定胆道闭锁患儿手术前、后血清sICAM 1、IL 18水平。结果　胆道闭锁患儿血清sICAM 1、IL 18水平明显高于健康对照组 ,且两者呈正相关。Kasai手术后两者水平比术前无明显下降 ,且黄疸越重 ,水平越高。结论　sICAM 1、IL 18参与胆道闭锁的进行性损伤的病理过程 ,手术后两者增高表明外科手术不能完全阻止BA的炎症 ,持续增高提示预后不良     

胆道闭锁预后相关因素的多中心研究

目的:应用多中心数据研究影响胆道闭锁预后的因素。方法:回顾性分析中国大陆地区11个儿童医学中心2015年6月至2017年6月569例胆道闭锁行Kasai手术,且术后满2年患儿的病历资料。分别记录患儿性别、手术日龄、术前肝功能指标、巨细胞病毒感染情况(血清巨细胞病毒IgM检测结果)、术后有无胆管炎的发生、术后胆管炎发生的...     

胆道闭锁Kasai术后相关预后因素分析和对策

目的 探讨影响胆道闭锁(biliary atresia,BA) Kasai术后预后的相关因素,了解我省BA的治疗现状.方法 回顾性分析2009年1月至2015年12月间,在山西省儿童医院行腹腔镜探查、胆道造影确诊胆道闭锁后行Kasai术且随访资料完整的91例患儿.采用Kaplan-Meier法计算各亚组患儿自体肝存活率.各亚组之间自体肝存活率的比较应用Log-rank检验,多因素分析采用COX回归模型.结果 全组患儿6个月、1年、2年的自体肝存活率分别为76.9％(70/91)、48.3％(44/91)、36.3％(33/91).按手术日龄将患儿分为:＜60 d(34例)、60～90(含60)d(47例)、90～120(含90)d(8例)和≥120 d(2例)组,各组2年累计自体肝存活率分别为55.8％(19/34)、44.7％(21/47)、12.5％(1/8)和0(P=0.047).男、女童2年累计自体肝存活率分别为36.4％(16/44)和46.8％(22/47),差异无统计学意义(P=0.313).有胆管炎发作组(67例)与无胆管炎发作组(24例)的2年累计自体肝存活率分别为31.3％(21/67)和62.5％(15/24)(P=0.011);黄疸消退明显组(61例)和黄疸消退不明显组(30例)的2年累计自体肝存活率分别为50.8％(31/61)和23.3％ (7/30) (P=0.012);肝功能恢复良好组(53例)和肝功能恢复较差组(38例)2年累计自体肝存活率分别为56.6％(30/53)和21.1％(8/38)(P=0.01);Ⅰ型、Ⅱ型、Ⅲ型闭锁的2年累计自体肝存活率分别为77.8％(7/9)、66.7％(4/6)和31.6％(24/76)(P=0.023),差异均有统计学意义.结论 手术日龄、有无胆管炎发作、黄疸消退情况、肝功能恢复情况及胆道闭锁分型为影响Kasai手术预后的影响因素,其中手术日龄、黄疸消退和肝功能恢复情况是影响生存的独立预后因素.提高民众对胆道闭锁的认知水平,加强术后随访评估,对提高胆道闭锁生存率有重要意义.     

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）肝脏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 ２７例ＣＢＡ患儿中,肝内毛细胆管发育良好为主１３例,其中１２例术后生存,生存率为９２．３１％;毛细胆管发育不良为主的１４例中,仅有４例生存,生存率为２８．５７％,两组生存率有显著性差异（     

胆道闭锁与病毒感染和免疫系统反应的相关研究

胆道闭锁的病因目前仍不清楚,了解其发病机制对于临床诊断及治疗具有至关重要的作用。近10年来,胆道闭锁可能是由病毒直接侵袭,或由病毒感染激发免疫异常所致的观点渐为小儿外科界所重视。病毒学研究发现,胆道闭锁可能与巨细胞病毒、轮状病毒或呼肠病毒等的感染有关。病理学研究提示,T细胞免疫反应很可能在胆道闭锁的发病中扮演了重要角色。该文总结了病毒学研究的现状,并从免疫调节基因、免疫细胞、炎症因子及自身免疫几方面阐述免疫系统反应研究的进展。     

胆道闭锁肝内iNOS及其调控因子异常表达与进行性肝损伤的相关性

目的 研究胆道闭锁(BA)患儿肝内诱导型一氧化氮合酶(iNOS)及其上下游调控因子表达情况,并探讨其与BA进行性肝损伤发生的关系.方法 应用免疫组织化学染色法对2002年10月至2007年3月在本院行Kasai手术的38例BA患儿与16例对照儿童肝组织iNOS表达情况进行研究;应用ELASA法对BA患儿和对照组外周血总一氧化氮(NO)代谢产物浓度进行测定;应用TUNEL法对BA患儿和对照组肝内胆管上皮凋亡指数进行测定;应用免疫印记法对BA患儿和对照组肝组织NF-κB表达进行半定量分析.结果 iNOS在BA患儿肝组织异常高表达,强度为0.30±0.08,而在对照组肝组织不表达.BA患儿外周血总NO代谢产物浓度为(90.40±12.46)mol/L,明显高于对照组的(63.67±5.78)μmol/L,且BA组总NO代谢产物浓度与血清ALT水平[(152.76±29.59)U/L]呈强正相关(r=0.97).BA组肝内胆管上皮凋亡指数(54.00±11.67)%远高于正常对照组(20.72±5.63)%,且与肝组织iNOS表达强度呈强正相关(r=0.99).NF-κB出在BA患儿肝组织中的表达(0.74±0.06)明显高于正常对照组(0.22±0.03),且与iNOS表达强度呈强正相关(r=0.97).结论 iNOS异常高表达在BA患儿进行性肝损伤中发挥重要作用,该作用是由iNOS及其上下游调控因子NF-κB、NO共同作用所实现.     

胆道闭锁胆汁成分与预后的关系研究

目的 探讨胆道闭锁术中胆汁成分变化与预后的关系。方法 本组对62例BA患儿在Kasai术中收集从肝门流出的胆汁,以25例胆总管囊肿患儿术中收集的胆汁作为对照组。采用全自动生化仪检测胆汁中总蛋白(TP)、胆汁酸(TBA)、总胆红素(TBIL)、直接胆红素(DBIL)、总胆固醇(CHO)、谷草转氨酶(AST)、谷丙转氨酶(ALT)、谷氨酰胺转肽酶(GGT)、碱性磷酸酶(ALP)的浓度。术后随访2～16个月,随访内容有黄疸消退时间,术后血生化,术后胆管炎发生情况及术后1年存活情况。结果 BA组胆汁中ALT(210.13±127.75) U/L、GGT(319.03±206.73) U/L、TP(45.86±18.16)g/L、CHO (0.08±(0.04) mmol/L浓度显著高于对照组,ALT:(69.39±44.55) U/L; GGT:(73.22±99.29)U/L; TP:(21.78±15.36)g/L; CHO:(0.02±0.03) mmol/L(P＜0.05); BA组胆汁中TBA( 189.81±107.81) μmol/L和DBIL (61.31±25.53 )μmol/L浓度显著低于对照组TBA:(735.06±199.75)μmol/L; DBIL:(90.43±27.18)μmol/L(P=0.000和P=0.035)。术前肝功能较好组胆汁中ALT(86.24±65.63) U/L、AST(801.64±516.50) U/L和GGT (205.82±112.25) U/L 浓度显著低于术前肝功能较差组,ALT:(244.67±175.69) U/L; AST:(1409.52±927.05) U/L;GGT:(362.05±235.57)U/L(P＜0.05);术前肝功能较好组胆汁中TBA (346.04±279.33)μmol/L 浓度显著高于术前肝功能较差组(192.47±93.55)μmbl/L(P=0.029)。术后3个月黄疸消退者胆汁TBA显著高于未消退者(251.18±131.83)μmol/L比(121.43±80.42) μmol/L,(P=0.007);术后3个月黄疸消退者胆汁DBIL(72.08±32.51 )μmol/L显著高于未消退者(40.22±23.47) μmol/L (P=0.022)。术后1年存活者胆汁TBA浓度较术后1年死亡者显著增高(258.63±105.63)μmol/L比(110.63±46.08) μmol/L,P= 0.015）;术后1年存活者胆汁ALT浓度较术后1年死亡者显著降低(8.72±8.83)U/L比(95.15±44.18)U/L,P=0.048。结论 BA术中胆汁成分与预后有明显的关系,胆汁中TBA较高、DBIL较高的BA患儿术后3个月黄疸消退较好;胆汁中TBA较高、ALT较低的BA患儿术后1年存活情况较好。胆汁TBA和ALT浓度可作为评价BA短期预后和中期预后的可靠指标。     

胆道闭锁异常肝组织蛋白筛查及预后相关性研究

目的 初步鉴定胆道闭锁肝组织异常蛋白表达情况,寻找与胆道闭锁发病和预后有关的差异表达蛋白.方法 用固相pH梯度双向凝胶电泳分离胆道闭锁和正常肝脏组织总蛋白,银染显色,Melanie3.02 2D图像分析软件分析,对部分蛋白质点用基质辅助激光解析电离飞行时间质谱(MALDI-TOF-MS)进行鉴定,查询数据库鉴定差异蛋白质.结果 获得了背景清晰、分辨率和重复性较好的双向凝胶电泳图谱,蛋白质匹配率达800%以上.胆道闭锁和正常组比较发现表达量变化达2倍以上的蛋白点有33个,表达量变化达4倍以上者有8个.胆道闭锁预后较好者和预后不好者比较发现表达量变化达4倍以上的蛋白点有22个,表达量变化达5倍以上者有18个.质谱鉴定其中15个点,其中7个点鉴定成功,分别是Viperin、SARM1、GPC3、APC、THUM2、MIA3和KIAA0649.结论 本研究成功鉴定出部分与胆道闭锁发病和预后有关的蛋白质,为进一步研究胆道闭锁致病机制和提高手术预后提供了新的研究方向,有进一步研究的价值.

Abstract：

Objective To investigate the expression of hepatic protein in patients with biliary atresia(BA), and find the relationship between the significant protein and prognosis of biliary atresia.Methods Immobile pH gradients isoelectric focusing was used as 1D, and vertical SDS-PAGE as 2D.Sliver staining, Melanie3. 02 2D analysis software, matrix assisted laser desorption ionization-time of flight mass spectrometry (MALDI-TOF-MS) and NCBInr database searching were used to separate and identify the proteome from liver in patients with biliary atresia. Results Satisfactory 2DE patterns were obtained. Thirty-three protein spots were remarkably changed in patients with biliary atresia compared with the control group. Twenty-two protein spots were significantly difference between BA patients with good prognosis and BA patients with poor prognosis. Fifteen protein spots were referred to mass spectrometry, and 7 protein spots were identified, including Viperin, SARM1, GPC3, APC,THUM2, MIA3 and KIAA0649. Conclusions In this study, proteins related to the prognosis and pathogenesis of biliary atresia were identified, which may contribute to prognosis and pathogenesis of biliary atresia.     

胆道闭锁肝纤维化与血清学监测

胆道闭锁（Biliary Artesia,BA）是新生儿阻塞性黄疸最常见的原因之一。其病理特征为胆管的进行性炎症和肝纤维化,发展速度快,涉及肝内外胆管,虽然通过Kasai手术（即肝门空肠吻合术）能够改善肝内外胆道的梗阻。但多数患儿仍发生进行性肝内胆管破坏和肝纤维化。最后发展为肝硬化和门静脉高压。晚期病例肝脏移植将不可避免。BA病人的预后受多种因素的影响,如Kasai手术时的年龄、病变的类型以及肝纤维化程度等。预测BA的预后以及判断肝纤维化程度。对选择肝移植的手术时机有着极其重要的意义。     

Bleeding as the first symptom of extrahepatic biliary atresia

Four cases of extrahepatic biliary atresia are reported. Each of these infants presented with haemorrhagic phenomena rather than with prolonged jaundice. The increased bleeding tendency was due to a vitamin K deficiency, probably caused by cholestasis-induced malabsorption. Therefore extrahepatic biliary atresia should be considered in each infant with a bleeding diathesis.Abbreviations Hb haemoglobin - SGOT aspartate transaminase - SGPT alanine aminotransferase - ^99mTc-IDA ^99mTechnetium-labelled iminodiacetic acid     

新生儿肝外胆道囊性病变的诊断与治疗

目的了解新生儿肝外胆道囊性病变的不同类型及其临床特点，掌握其鉴别诊断的要点。方法分析9例新生儿肝外胆道囊性病变患儿的临床表现、肝功能、B超、CT、光镜、术中胆道造影的资料。结果本组胆道闭锁和新生儿胆总管囊肿女性多见，以梗阻性黄疸为主要表现，肝功能检查结果提示梗阻性黄疸。二者肝脏的组织学改变相似。但B超和CT检查发现，胆道闭锁组囊肿与胆囊大小均小于胆总管囊肿组。术中胆道造影显示，Ⅰ型胆道闭锁组肝内胆管变形、连续性中断；Ⅲ型胆道闭锁肝内胆管无法显示；胆总管囊肿组肝内胆管形态正常或略有被动扩张。结论多种胆道畸形都可以表现为新生儿肝外胆道囊肿，B超和术中胆道造影可以帮助鉴别这类疾病并且为治疗提供帮助。     

新生儿肝外胆道囊性病变的诊断与治疗

目的了解新生儿肝外胆道囊性病变的不同类型及其临床特点,掌握其鉴别诊断的要点。方法分析9例新生儿肝外胆道囊性病变患儿的临床表现、肝功能、B超、CT、光镜、术中胆道造影的资料。结果本组胆道闭锁和新生儿胆总管囊肿女性多见,以梗阻性黄疸为主要表现,肝功能检查结果提示梗阻性黄疸。二者肝脏的组织学改变相似。但B超和CT检查发现,胆道闭锁组囊肿与胆囊大小均小于胆总管囊肿组。术中胆道造影显示,Ⅰ型胆道闭锁组肝内胆管变形、连续性中断;Ⅲ型胆道闭锁肝内胆管无法显示;胆总管囊肿组肝内胆管形态正常或略有被动扩张。结论多种胆道畸形都可以表现为新生儿肝外胆道囊肿,B超和术中胆道造影可以帮助鉴别这类疾病并且为治疗提供帮助。     

测定胆道闭锁术后皮肤胆红素动态变化与预后的关系

目的 初步探讨胆道闭锁患儿术后测定皮肤胆红素动态变化与黄疸消退及预后的关系.方法 2009年7月至12月,收治黄疸患儿52例,男19例,女33例,年龄36 d～304 d,平均年龄83 d,包括胆汁淤积5例,胆道闭锁47例,采血测血清胆红素同时测其皮肤胆红素.同期收治非黄疸患儿59例,男28例,女31例,年龄26 d～200 d,平均年龄119 d,采血测血清胆红素同时测其皮肤胆红素.其中行Kasai手术胆道闭锁患儿23例,男8例,女15例,年龄36d～127d,平均年龄63d,测其住院期间每日皮肤胆红素并以其术后3个月血清总胆红素20μmol/L为界,将其分为黄疸是否消退两组,对比观察两组患儿住院期间皮肤胆红素及其下降水平.结果 皮肤胆红素(x)与血清总胆红素(y)呈线性回归关系,其回归方程为:y=0.945x-46.273(P＜0.05).正常儿童的皮肤胆红素95%参考值范围为33.14～96.14μmol/L.黄疸消退患儿术后11 d、12 d皮肤胆红素水平(159 μmol/L和151μmol/L)显著低于黄疸未消退患儿(205 μmol/L和210 μmol/L),差异有统计学意义(P＜0.05).黄疸消退患儿术后平均每天皮肤胆红素下降较快(5.04 μmol/L vs 2.33 μmol/L,P＜0.05).结论 测定皮肤胆红素是一种安全可靠的方法,可以较早反映胆道闭锁患儿术后黄疸消退情况.

Abstract：

Objective To study the relationship between the post-op transcutaneous bilirubin changes after Surgery and the prognosis in children with biliary atresia. Methods Between July 2009to December 2009,52 children( 19 males and 33 female, age: 36 d～304 d, the mean age 83d, including 5cholestasis and 47 biliary atresia)with jaundice and 59 non-jaundiced children's (28 males and 31 females,aged 26 d～200 d and the mean age 119 d) were recruited for the study. The transcutaneous bilirubin and total serum bilirubin of children with jaundice and non-jaundice were measured at the same time. Twenty-three children with biliary atresia (8 males and 15 females, aged 36 d～ 127 d and the mean age 63 d) underwent Kasai operation. The children were divided into good or poor prognosis groups according to the serum bilirubin levels of 20 μmol/L three months after operation. Measurements were compared between the two groups during hospital stay. Results Linear regression oftranscutaneous bilirubin (x) correlated with that of serum bilirubin (y) (y = 0. 945x - 46. 273, P＜0. 05). The 95% confidence interval of transcutaneous bilirubin in normal children was 33. 14～96. 14μmol/L. The transcutaneous bilirubin of children with good prognosis was significantly lower than that of children with poor prognosis on postoperative day 11 ( 159 μmol/L vs 205 μmol/L,P＜0. 05) and 12(151 μmol/L vs 210μmol/L,P＜0. 05). Postoperative average daily decline of transcutaneous bilirubin in children with good prognosis was faster (5. 04 μmol/L vs 2. 33 μmol/L, P＜0. 05). Conclusions Transcutaneous bilirubin measurement is a safe and reliable method of monitoring the decline of jaundice of children with biliary atresia.     

A case of biliary atresia with cystic dilatation of the extrahepatic bile duct and polysplenia syndrome

A 79-day-old girl presented with jaundice and acholia. Laboratory findings disclosed elevated levels of bilirubin, transaminases, and gamma-glutamyl transferase. A chest X-ray film showed dextrocardia. A computed tomographic scan revealed a cystic mass at the porta hepatis, multiple spleens in the right side of the abdomen, and absence of the inferior vena cava. Under the diagnosis of biliary atresia (BA) (I-cyst) associated with polysplenia syndrome, a hepatic portojejunostomy was performed at 80 days of age. The postoperative course was uneventful, and the jaundice cleared. Although the occurrence of associated anomalies in BA is rare, polysplenia is most commonly seen, and its incidence is reported to be 2% to 10%. Patients with BA and polysplenia usually have a poor prognosis, however, this patient may have a good prognosis due to uneventful recovery from the jaundice. Accepted: 30 August 1999     

胆道闭锁术时年龄及肝脏病理与近期预后的相关性研究

目的 研究胆道闭锁患儿Kasai手术术时年龄、肝脏病理与近期预后之间的相关性.方法 对2008年7月至2011年6月收治于我院普外科的胆道闭锁患儿进行Kasai术后6个月的近期预后随访,分为优秀组、良好组和不良组.每组随机抽取20例共计60例作为样本.回顾样本病历资料获取术时年龄.获取样本病例Kasai手术术中取得并保存的肝脏病理标本,应用Masson染色评定肝纤维化程度;应用CK19免疫组化染色和图像分析技术评定胆管反应程度.统计学分析术时年龄、肝脏病理学指标和近期预后之间的相关性.结果 优秀组、良好组和不良组的平均术时年龄分别为(77.55±24.64)d、(90.30±24.13)d和(72.35±24.53)d,无术时年龄＜30d病例;优秀组、良好组和不良组的无肝硬化病例和肝硬化病例分别为11:9、11:9和16:4;优秀组、良好组、不良组平均胆管面积比例分别为(5.62±3.62)％、(8.50±4.08)％和(5.15±3.77)％.不同预后病例术时年龄、肝脏病理学指标均无统计学差异;肝硬化组较无肝硬化组术时年龄无统计学差异,肝硬化组较无肝硬化组胆管反应程度严重;术时年龄与胆管反应呈显著线性正相关.结论 胆道闭锁患儿Kasai术后6个月近期预后和术时年龄、肝纤维化程度、胆管反应程度均无明显相关性,近期预后可能受术者经验和手术技巧影响;术时年龄越大胆管反应越重,胆管反应对肝纤维化有促进作用,尽早手术对改善远期预后有帮助.     

胆道闭锁肝胆病理改变与预后的关系研究

目的 通过研究BA患儿术中肝活检和肝门纤维块的病理表现,探讨肝脏和纤维块病理表现与BA预后的关系.方法 2009年4月至2010年9月,61例BA患儿在我院行Kasai手术治疗,术中取肝活检和肝门纤维块.病理切片观察肝小叶结构、纤维化情况,将所有BA患儿根据肝病理诊断是否为胆汁性肝硬化分为肝硬化组和非肝硬化组.根据镜下观察到纤维块中小胆管增生的程度分为少许小胆管增生组和较多小胆管增生组.术后随访3～17个月.结果 病理诊断为肝硬化者术时年龄较大、术中门脉测压较高、术后肝功能恢复情况较差.肝硬化组术后3个月黄疸消退率明显较非肝硬化组低(X2=4.020,P=0.045),术后1年病死率明显较非肝硬化组高(P=0.046).肝硬化者纤维块内小胆管增生程度较重(x2=4.244,P=0.039);纤维块内小胆管增生越严重,术后3个月黄疸消退率越低(P=0.045).结论 肝病理检查是较早的评估BA患儿预后的可靠方法,肝硬化是影响BA预后的重要因素;纤维块小胆管增生程度与BA患儿的短期预后有相关关系,但其是否与BA的远期预后有关系还需要进一步的研究.

Abstract：

Objective To investigate the correlation between the pathologic changes of liver and fibrous portal tissue and prognosis of biliary atresia (BA). Methods Between April 2009 and September 2010, the Kasai's procedures were carried out on 61 patients with BA. Specimens of the liver tissue and fibrous portal tissue were collected in operation. The pathology of the liver tissue and fibrous portal tissue was studied. Based on the severity of hepatic cirrhosis, the patients were grouped into two groups: cirrhosis group and no cirrhosis group. According to whether or not there was obvious bile ductular proliferation in fibrous portal tissue, the hepatic cirrhosis patients were further grouped into 2 groups. All of patients with BA were followed up for 3 to 17 months. Results The patients of hepatic cirrhosis group were older at operation, and had higher portal pressure and poorer prognosis. The 3 months post-operative jaundice clearance rate of cirrhosis patients was significantly lower than that of no cirrhosis patients (39. 5% vs 83. 3%,X2 = 4. 020,P= 0. 045). The 1-year post-operative mortality of the cirrhosis patients was significantly higher than that of no cirrhosis patients (53. 3% vs 0,P= 0. 046). The patients of the cirrhosis group had more bile ductular proliferation (60% vs 0, X2 = 4. 244, P = 0. 039). The more severe bile ductal proliferation in the cirrhosis patients was, the lower jaundice clearance rate they would have (76. 5% vs 38. 5%,P=0. 045). Conclusions The pathologic findings of liver can be used to predict the prognosis of the BA patients after Kasai's procedure. The patients with severe hepatic cirrhosis and bile ductal proliferation have poor prognosis.     

Bone density and 25-hydroxyvitamin D level in extrahepatic biliary atresia

Biliary atresia (BA) represents a common cholestatic affliction of the gastrointestinal tract affecting infants and children. The objective of the present study was to evaluate 42 patients (20 with and 22 without jaundice) diagnosed with extrahepatic BA for bone mineral content and serum 25-hydroxyvitamin D (HVD) levels. Physical examination and anthropometric nutritional assessment were performed. The investigation included liver function tests and serum calcium (Ca), phosphate (P), magnesium (Mg), and 25-HVD levels. Dual-energy X-ray absorptiometry was used to measure the bone mineral density (BMD) of the lumbar spine (L₁–L₄). Our results showed that 16 jaundiced␣patients (80%) and only 3 nonjaundiced patients (13.6%) showed osteoporosis (P< 0.05). All patients had normal serum Ca and P levels. Only 1 nonjaundiced patient had a low serum Mg level. Serum 25-HVD levels (mean ± SD) were 20.71 ± 8.24, 16.12 ± 4.3, and 9.18 ± 5.84 ng/ml, respectively, in subjects with normal bone density (n=7), osteopenia (n=3), and osteoporosis (n=11). Bone disease represents a well-known complication among long-term survivors of BA. To date, the pathogenesis has remained unexplained. Since, as demonstrated in the present study, jaundiced patients develop osteoporosis more frequently than nonjaundiced patients, hyperbilirubinemia may have an influence. Bone-mineral deficiency can be detected earlier by means of BMD measurement (non-invasive method) than by measuring serum Ca, P, and Mg levels in these patients. Accepted: 27 November 2000