先天性胆总管囊肿致肝脏病损的临床分析及转归

为了对儿童先天胆总管囊肿（ＣＢＤ）导致肝脏病损的有关临床因素及其转归进行分析， 对收治的３７例先天性胆总管囊肿患儿按肝脏病理改变的程度分成三组：肝硬变组１５例，肝硬变前期组２０例及正常肝细胞组２例。结果，肝硬变组年龄明显小于肝硬变前期组，前者平均年龄１７．３７个月，后者为７０．２５个月（Ｐ＜０．００１）。肝硬变组患儿均有持续性贡疽史，肝硬变前期组有持续性黄疸史的仅３例，肝硬变组囊肿直径平均值为７．８ｃｍ，肝硬变前期组囊肿直径平均值为４．７ｃｍ（Ｐ＜０．０５）。肝硬变组９例获术后长期随访，８例恢复满意。结论：①ＣＢＤ患儿出现症状越早，肝硬变机会越大。　②胆道梗阻是ＣＢＤ导致肝脏病损的主要原因。　③ＣＢＤＩ型患儿囊肿越大，对胆流动力学影响越大，　对肝脏的影响也越大。　④对已出现严重肝硬变的ＣＢＤ患儿仍应持积极态度，胆道梗阻解除后肝硬变仍有逆转的可能。     

感染新生儿中性粒细胞CD11b表达

目的 探讨新生儿细菌感染中性粒细胞（ＰＭＮ）ＣＤ１１ｂ表达的关系。方法 采用全血流式细胞术和直接免疫荧光法检测１０例感染新生儿中性粒细胞ＣＤ１１ｂ平均荧光强度（ＭＦＩ）１１例正常新生儿作为对照。结果 感染新生儿ＰＮＭ ＣＤ１１ｂ ＭＦＩ显著低于正常新生儿（Ｐ＝０．００３）。高ＣＲＰ组ＣＤ１１ｂ ＭＦＩ低于低ＣＲＰ组（Ｐ＝０．０１６）。结论 感染新生儿ＰＭＮ ＣＤ１１ｂ表达下调，其下调与感染严重度有     

射频消融对兔心内膜单相动作电位影响的实验研究

目的探讨射频导管消融（ＲＦＣＡ）对心内膜单相动作电位（ＭＡＰ）的影响，为进一步完善ＲＦＣＡ的过程提供理论依据。方法选取健康家兔３６只，用接触电极导管监测ＲＦＣＡ前后兔心内膜的ＭＡＰ变化并观察ＲＦＣＡ不同时间（１０～６０秒）和不同温度（４０～９０℃）水平ＭＡＰ的变化。结果（１）单相动作电位振幅（ＭＡＰＡ）和０相最大上升速度（Ｖｍａｘ）随ＲＦＣＡ时间延长及温度升高而降低或下降，持续放电３０秒或温度达５０℃以后ＭＡＰＡ及Ｖｍａｘ几乎不再变化；（２）整个过程的单相动作电位时程（ＭＡＰＤ）无明显变化且未见心律失常发生。结论（１）当ＲＦＣＡ温度达５０℃心肌电活动以及心肌组织已经产生有效的损伤；ＲＦＣＡ对ＭＡＰＤ不产生影响，故不易导致心律失常（２）将ＭＡＰ记录与温度监测结合起来，对判断ＲＦＣＡ前导管心肌的接触及ＲＦＣＡ后心肌的损伤有一定的指导意义     

先天性胆总管囊肿合并门脉高压症

目的 探讨先天性胆总管囊肿（ＣＣ）合并门脉高压的原因、类型及转归。方法 将１７例ＣＣ合并门脉高压与１３例ＣＣ不合并门脉高压者进行比较,项目包括病程、胆源性发热、胆总管囊肿的最大前后径、胆道压力、肝组织病理变化、病理图像分析,对门脉高压患儿进行随访。结果 门脉高压组（ＰＨ）胆源性发热发生率高,胆道压力高于非门脉高压组（ＮＰＨ）,门脉高压组肝组织病理观察发现：１４例肝小叶完整,门静脉及肝静脉分支走行正     

早搏与心功能的关系及影响因素的研究

为探讨小儿早搏对心功能的影响，采用多普勒超声心动图测定４０例早搏患儿的单个早搏射血分数（ＰＢＥＦ）、心脏指数（ＰＢＣＩ）；单个非早搏的射血分数（ＮＰＢＥＦ）、心脏指数（ＮＰＢＣＩ）及实际的射血分数（ＡＥＦ）、心脏指数（ＡＣＩ）。同时研究这些指标与心电图和心肌酶的关系。结果显示：（１）早搏患儿ＰＢＥＦ、ＰＢＣＩ均小于ＮＰＢＥＦ、ＮＰＢＣＩ；病程长者ＰＢＥＦ、ＡＥＦ及ＡＣＩ均下降；室性早搏者的ＡＣＩ减少；早搏＞１０次／分者的ＡＥＦ及ＡＣＩ下降；Ｒ－Ｒ′／Ｒ－Ｒ比值小者的ＰＢＥＦ、ＡＥＦ及ＰＢＣＩ亦小；早搏ＱＲＳ－Ｔ综合波长者的ＰＢＥＦ及ＰＢＣＩ减少。（２）肌酸磷酸激酶同功酶（ＣＫ－ＭＢ）升高者的ＰＢＥＦ、ＡＥＦ、ＰＢＣＩ、ＡＣＩ均值的下降比ＣＫ－ＭＢ不升高者显著。研究提示，早搏＞１０次／分、Ｒ－Ｒ′／Ｒ－Ｒ＜０．６、ＱＲＳ－Ｔ综合波＞０．４秒和ＣＫ－ＭＢ＞１６ＩＵ／Ｌ者的心功能多受到影响，应予以积极治疗。     

儿童白血病脑脊液P~(53)蛋白和ras癌基因蛋白表达及临床意义探讨

为探讨Ｐ(５３)蛋白和ｒａｓ癌基因蛋白在白血病儿童脑脊液（ＣＳＦ）中的表达水平及与白血病化疗的关系,应用Ｗｅｓｔｅｒｎ斑点印迹法检测５４例儿童急性白血病ＣＳＦ中Ｐ(５３)蛋白和ｒａｓ癌基因蛋白表达水平。结果显示：白血病儿童危象期ＣＳＦ中Ｐ(５３)蛋白明显高于非肿瘤对照水平（Ｐ＜０．０１）。缓解期Ｐ(５３)蛋白和ｒａｓ癌基因蛋白与危象期比有增高趋势（Ｐ＜０．０１）,同时高于非肿瘤对照水平（Ｐ＜０．０１）。白血病儿童ＣＳＦ治疗前Ｐ(５３)／ｒａｓ比值明显升高（Ｐ＜０．０１）,缓解期比值下降接近非肿瘤对照组（Ｐ＞０．０５）。１７例白血病儿童化疗前后双份ＣＳＦ中Ｐ(５３)／ｒａｓ比值动态观察发现,化疗前比值升高,化疗后比值下降,其临床表现及预后好,如化疗前后比值持续在高水平不下降,临床表现差,预后不佳,骨髓近期将复发。提示：检测急性白血病儿童ＣＳＦ中Ｐ(５３)蛋白和ｒａｓ癌基因蛋白,计算Ｐ(５３)／ｒａｓ比值变化有助于指导化疗、评定疗效、监测预后。     

干扰素剂量和给药方法对婴幼儿呼吸道合胞病毒肺炎疗效的影响

研究目的探讨干扰素剂量和给药方法对婴幼儿呼吸道合胞病毒肺炎疗效的影响。研究方法婴幼儿呼吸道合胞病毒（ＲＳＶ）肺炎４８例，随机分为Ａ、Ｂ、Ｃ、Ｄ４组，每组１２例。各组基础治疗相同，Ａ组肌注大剂量α干扰素［α－ＩＦＮ，５×１０４ＩＵ／（ｋｇ·ｄ）］。Ｂ组肌注小剂量α－ＩＦＮ［２×１０４ＩＵ／（ｋｇ·ｄ）］，Ｃ组肌注小剂量α－ＩＦＮ加雾化吸入小剂量γ－ＩＦＮ（３×１０３ＩＵ／次），Ｄ组雾化吸入小剂量γ－ＩＦＮ．结果Ｄ组患者退热时间比Ａ、Ｂ、Ｃ３组明显延长（Ｐ＜０．０１），其他症状（如咳嗽、呼吸困难、喘鸣、干、湿罗音等）消失时间Ａ与Ｃ组、Ｂ与Ｄ组相比均无显著差异（Ｐ＞０．０５），Ａ、Ｃ组与Ｂ、Ｄ组相比，有显著性差异，Ａ、Ｃ组显效较早。结论ＩＥＮ治疗ＲＳＶ肺炎的疗效与给药剂量和方法密切相关，小剂量ＩＦＮ治疗效果较差。小剂量α－ＩＦＮ肌注配合小剂量γ－ＩＦＮ雾化吸入治疗ＲＳＶ肺炎效果最好。     

重组干扰素α—2a治疗病毒性中枢神经系统感染前后体液干扰素水…

目的：探讨病毒性中枢神经系统感染时，血液和脑脊液干扰素（ＩＦＮ）变化情况，方法：７０例患者应用重组干扰素α－２ａ（ｒＨｕＩＦＮα－２ａ）治疗，采用微量细胞病变抑制法测定血液和脑脊液ＩＦＮ。结果治疗前血液ＩＦＮ测定阳性率为８４．３％（５９／７０）平均含量为８．４ＩＵ／ｍｌ。治疗后２不时内－ＩＦＮ均呈阳性，平均含量升至２６．８ＩＵ／ｍｌ（Ｐ〈０．０１）；脑脊液ＩＦＮ变化轻微，阳性率由６１．４％，（４３     

特发性血小板减少性紫癜患儿T细胞免疫功能的变化

目的 探讨Ｔ淋巴细胞亚群、白细胞介素１０（ＩＬ－１０）、γ－干扰素（ＩＦＮ－γ）在特发性血小板减少性紫癜（ＩＴＰ）中的变化及其用地塞米松（ＤＥＸ）治疗的影响。方法 采用ＤＥＸ治疗ＩＴＰ患儿,分别于治疗前后采取静脉血标本,应用改良碱性磷酸酶－抗碱性磷酸酶（ＡＰＡＡＰ）法及酶标记免疫吸附测定（ＥＬＩＳＡ）法分别检测Ｔ细胞亚群及血清ＩＬ－１０、ＩＦＮ－γ的含量。结果 ＩＴＰ患儿ＤＥＸ治疗前外周血ＣＤ４＾     

哮喘患儿血浆和淋巴细胞诱生γ－干扰素水平的检测及其意义

应用生物素－亲合素双抗体夹心酶联免疫吸附分析技术（ＡＢＣ－ＥＬＩＳＡ）检测了２４例哮喘患儿血浆和外周血单个核细胞（ＰＢＭＣ）诱生γ－干扰素（ＩＦＮ－γ）水平。结果显示：患儿血浆ＩＦＮ－γ水平极低（１．３３±０．０８μｇ／Ｌ），经植物血凝素（ＰＨＡ）刺激后，ＰＢＭＣ诱生ＩＦＮ－γ水平仍明显低于正常对照组（Ｐ＜０．００１）。哮喘患儿血清总ＩｇＥ水平明显升高。用麻疹疫苗治疗后ＩＦＮ－γ诱生水平无明显变化。提示患儿体内细胞因子产生失衡，ＩＦＮ－γ产生减少与哮喘的发病、病程和易诱发病毒反复感染有一定关系。     

临时性门腔静脉架桥联合流出道改良重建在部分供肝肝移植中应用的实验研究

目的 通过动物肝移植实验,探索门腔静脉架桥和改良肝静脉出口重建这一联合方法的可行性及价值.方法 选择20～25 kg和10～15 kg健康杂交犬各12只,组成供体组和受体组,并随机配对.供体手术取左外侧叶及左中央叶为供肝,然后完全阻断门静脉.受体犬先预置门腔静脉之间端侧吻合架桥的分流通道,切肝门静脉阻断时开放,供肝植入...     

Development of liver size and perfusion after reduced-size liver transplantation in children

The technique of segmental liver transplantation (s-LTx) provides a method to overcome the shortage of suitable livers for small recipients. Patient survival rates are parallel to those obtained with whole liver transplantation (w-LTx). For long-term rehabilitation, adaptive liver growth and adequate perfusion is crucial; however, morphometric and hemodynamic parameters in growing children with s-LTx are not available. Seventeen children who received a s-LTx and 25 with a w-LTx who had follow-up evaluation 1 and 2 yr after LTx were studied. Mean age at time of transplantation was 4.3 +/- 3.5 yr for s-LTx and 10.3 +/- 6.0 yr for w-LTx, mean height 98 +/- 21 cm and 122 +/- 30 cm respectively. At follow-up evaluation mean values for liver enzymes, bilirubin and prothrombin time were in the normal ranges for both groups. Liver dimensions were measured by gray scale ultrasound, and hemodynamic parameters by Doppler sonography in the portal vein and hepatic artery using an Acuson 128 machine. Maximal (Vmax), minimal (Vmin) and time-average velocity (TAV) were measured and the resistive index (RI) calculated. We found that 1 and 2 yr after LTx liver dimensions were at a mean in the upper normal range of healthy controls. Spleen size was above the normal range and did not show any tendency towards regression. Mean Vmax in the hepatic artery in s-LTx and w-LTx was 48 cm/sec vs. 28 cm/sec after 1 yr and 30 cm/sec vs. 35 cm/sec after 2 yr, the RI 0.66 vs. 0.55 and 0.59 vs. 0.73, respectively (p for all parameters > 0.05). Maximal portal vein flow was 25 cm/sec in s-LTx vs. 29 cm/sec in w-LTx. Blood flow calculated by vessel diameter and TAV showed no statistical difference between both groups. In conclusion, liver size after s-LTx and w-LTx was increased to the upper normal range, and portal vein blood flow velocities were within the normal range. Vmax in the hepatic artery was reduced in s-LTx; however, the reduction was to the same extent as in w-LTx. In the view of long-term functional adaptation, s-LTx is not inferior to w-LTx.     

D-dimer and portal vein status in splenectomized Egyptian β-thalassemia major patients: a prospective single-thalassemia center experience

Splenectomy is a recognized cause of portal vein thrombosis. Thirty-six β-thalassemia major (β-TM) patients were followed up for 36 months to evaluate changes in D-dimer levels (as a possible marker for thrombosis development) and portal vein status (by portal duplex ultrasound) at both early and late postlaparoscopic splenectomy periods. They were classified into group I if they were splenectomized in the study period (n = 12), or group II if they were splenectomized during the 5 years preceding the period (n = 24). In group I, D-dimer was measured 5 times: 1 day presplenectomy, the 1st week, 6th week, and 6th month postsplenectomy, and at the study end, whereas in group II, D-dimer was measured twice: at the study entry and end. Portal duplex was done 1 week postsplenectomy (group I) and at study end in both groups. Presplenectomy D-dimer levels in group I were significantly higher compared with the 6th month (P = .042) and study end (P = .03), whereas 1st week (postsplenectomy) D-dimer levels had a high mean of 3497.3 ng/mL, lowered at the 6th week (P = .017), at the 6th month (P = .008), and at study end (P = .005). D-dimer levels in group II showed no difference between study entry and end (P = .104). Portal vein "diameter and flow" were within normal findings in both groups. In this 3-year prospective study, a subclinical hypercoagulable state was detected 1 day prior to splenectomy and in the early postsplenectomy period, as evidenced by high D-dimer levels. Laparoscopic splenectomy was not associated with portal venous thrombosis either clinically or by duplex sonography.     

Changes in ovine hepatic circulation and oxygen consumption at birth

The umbilical vein provides the majority of hepatic blood flow during fetal life. After birth, liver blood flow is derived from the hepatic artery and portal vein, but ductus venosus patency can alter portal venous blood flow to the liver. To characterize changes in hepatic blood flow and oxygen metabolism in the immediate perinatal period, we studied liver and ductus venosus blood flow in seven fetal sheep before and after birth using the radionuclide-labeled microsphere method. Hepatic blood flow fell from 423 +/- 117 (mean +/- SD) mL/min/100 g liver in the fetus to 144 +/- 73 by 2 h after delivery. Although portal venous blood flow increased progressively from 2 to 10 h (137 +/- 48 to 305 +/- 140 mL/min/100 g), because of increasing ductus venosus shunt flow, total hepatic blood flow did not change. Hepatic arterial flow was 46 +/- 24 mL/min/100 g at 2 h, providing 35% of total hepatic blood flow, and did not change over the next 8 h. Hepatic oxygen delivery fell after birth from 58 +/- 25 mL/min/100 g liver in the fetus to 21 +/- 11 at 2 h and then remained constant. Hepatic oxygen consumption was 7.3 +/- 2.6 mL/min/100 g liver in the fetus and ranged from 3.2 +/- 1.5 to 4.1 +/- 1.8 mL/min/100 g liver during the 10 h after birth. Loss of the umbilical-placental circulation at birth substantially reduces hepatic blood flow. Hepatic arterial flow does not increase to compensate for decreases in total hepatic blood flow. The persistent ductus venosus shunt compromises portal venous supply to the liver.(ABSTRACT TRUNCATED AT 250 WORDS)     

Congenital portosystemic vascular malformations

Congenital portosystemic shunts are developmental abnormalities of the portal venous system resulting in the diversion of portal blood away from the liver to the systemic venous system. Such malformations are believed to come from an insult occurring between the fourth and eighth week of gestation during the development of hepatic and systemic venous systems, and could explain their frequent association with cardiac and other vascular anomalies. They are currently categorized into end-to-side shunts (type I) or side-to-side shunts (type II). This article aims to review the common symptoms and complications encountered in congenital portosystemic shunts, the surgical and endovascular treatment, and the role of liver transplantation in this disease. We will also focus on the current controversies and the areas where there is potential for future studies.     

Single pretransplant bolus of recombinant activated factor VII ameliorates influence of risk factors for blood loss during orthotopic liver transplantation

Large blood loss and transfusions during liver transplantation (LTx) may lead to serious complications and have a negative impact on post-transplant mortality and morbidity. In the retrospective study we compared two groups of recipients of primary cadaveric liver transplantation: group I (study group), consisted of 28 patients with preoperative risk of high intraoperative blood loss, including severe uncorrected coagulopathy. This group was given a bolus of recombinant activated factor VII (rFVIIa) just before LTx. Group II (control group) included 61 patients without a particular risk for increased intraoperative blood loss. These patients were not given rFVIIa. We analyzed both groups for: coagulation parameters before, during and after surgery (INR, APTT, factor VII activity), blood and FFP transfusions, operative time, postoperative complications (vascular thrombosis, reoperation for bleeding), postoperative ICU stay, post-transplant hospitalization time and mortality. Patients from the study group (I) had significantly worse coagulation parameters than patients in the control group (II) at the start of the surgical procedure; however, after administration of a bolus of rFVIIa there was immediate correction of coagulation in all recipients. No significant differences in intraoperative blood transfusions were observed between study and control groups (1980 +/- 311.4 mL vs. 1527 +/- 154.2 mL, respectively), operating time (8.7 h vs. 8.9 h) or ICU and hospital stay (7.03 days vs. 6.15 days and 40.89 days vs. 41.1 days). Re-exploration because of bleeding was performed in three patients from group I (10.7%) and in seven patients (11.5%) from group II. No single case of vascular thrombosis was observed in the study group, while in the control group there were three hepatic artery thromboses, two portal vein thromboses and one hepatic vein thrombosis. We conclude that rFVIIa given preoperatively to liver transplant recipients with several risk factors for high intraoperative bleeding adjusts these patients to a normal risk group, without an increased risk for thrombotic complications.     

Relationship between oxidative stress and antioxidant systems in the liver of patients with Wilson disease: hepatic manifestation in Wilson disease as a consequence of augmented oxidative stress

The role of oxidative stress in the pathogenesis of liver disease in Wilson disease (WD), a genetic disorder characterized by excess hepatic deposition of copper that generates free radicals, remains unclear. This study investigates oxidative stress on the liver and hepatic antioxidant responses in WD using liver specimens from affected patients showing mild liver damage (group I, n = 3), moderate or greater liver damage (group II, n = 5), and fulminant hepatic failure (group III, n = 5) and from asymptomatic carriers (n = 2). Decreased ratios of reduced glutathione (GSH) to oxidized glutathione (GSSG) and increased thiobarbituric acid reactive substance (TBARS), a lipid peroxidation product, were found in every affected patient, especially in group II and III patients. Activities and protein expressions of Mn-dependent superoxide dismutase (Mn-SOD), CuZn-dependent superoxide dismutase (CuZn-SOD), and catalase were decreased in all patients, especially in group III patients. Glutathione peroxidase (GPx) activity was decreased only in group III patients. Asymptomatic carriers without any clinical manifestations showed normal TBARS level and GSH/GSSG ratio with increases in both GSH and GSSG levels. Their CuZn-SOD, Mn-SOD, and catalase activities were increased. These results suggest that excessive copper-derived oxidants contribute to development and progression of liver disease in WD.     

Anemic necrosis of the liver after umbilical vein catheterization.

We report 22 cases of hepatic necrosis following catheterization of the umbilical vein. The morphology of the necrotic lesions, which were confined to the left lobe in 21 patients, was that of an anemic infarct. Their causes are multiple and include intimal damage during the insertion of the catheter with consecutive thrombosis of portal vein branches, the postpartum immaturity of the arterial hepatic oxygen supply, postpartum hypoxia due to respiratory failure of cerebral or pulmonary origin, obstruction of portal venous flow through the catheter as such, the postpartum involution of the left hepatic lobe, and prolonged periods of catheterization. Toxic damage of the hepatic parenchyma due to an infusion of Tris buffer through the catheter into the portal venous system is a potential additional factor.     

Rex手术治疗小儿肝外门静脉高压的应用进展

肝外门静脉梗阻(Extra-hepatic portal venous obstruction,EHPVO)是引起小儿门静脉高压的常见原因之一.研究发现,大约66％～76.5％的小儿门静脉高压是由EHPVO引起的,其中以门静脉海绵样变多见.Rex手术已经成为当前治疗小儿肝外门静脉高压的重要手术方式之一,由于其重建入肝血流,恢复门静脉解剖结构和生理功能的作用,是小儿肝外门静脉高压的根治性手术方法.     

Advanced biliary atresia : Is portoenterostomy justified in all infants?

During 1989–98, of the 127 patients with biliary atresia, 23 were seen with advanced biliary atresia (ABA) presenting with (i) at more than 120 days of age (ii) established cirrhosis and (iii) features of portal hypertension. Sixteen of these underwent exploratory laparotomy, dissection at the porta hepatis and hepatico-portoenterostomy (HPE) (group I). The remaining 7 infants underwent portal dissection and sump drainage only (instead of HPE). The drainage if any was evaluated for any change in colour, volume and concentration in the post-operative period (group II). Age and presentation were the same in both the groups. In group-II, HPE was considered only if the bile flow was noted after portal dissection. Bile flow was seen in 3/16 in group I and 0/7 in group II. The fall in serum bilirubin during the first seven post operative days was noted in 2/16 in group I and 0/7 in group II. No drain output was recorded in any of the group II infants. The incidence of complications and the duration of hospital stay was significantly higher in group I patients. The sump drainage as an alternative procedure to HPE not only served the purpose of evaluating the patients with ABA for the possible bile flow in the post operative period but also avoided the need for a major operative procedure like HPE.