肝功能检查在婴儿肝炎综合征和先天性胆道闭锁的鉴别诊断中的价值

目的：该文采用受试者工作特征曲线（ROC曲线）对肝功能检查中常用的7项血清学指标进行分析,探讨常用的肝功能检查在婴儿肝炎综合征（infantile hepatitis syndrome,IHS）和先天性胆道闭锁（biliary atresia,BA）中的鉴别诊断价值。方法：分别检测IHS组（n=103）和BA组（n=60）的丙氨酸转氨酶（ALT）、天冬氨酸转氨酶（AST）、γ-谷氨酰基转移酶（γ-GT）、碱性磷酸酶（ALP）、血清总胆红素（TB）、结合胆红素（CB）和血清白蛋白（ALB）等7个生化指标,应用ROC曲线进行分析,评价上述各指标的敏感性和特异性,阳性预测值和阴性预测值,并得到最佳临界值。对γ-GT,TB 和CB采用联合试验,提高诊断效率。结果：与IHS组相比,BA组的ALT、AST、γ-GT、TB 和CB水平显著升高（PCB>TB>AST;CB的阴性预测值最高,TB次之。CB的阴性似然比最低;CB的Youden指数最高,γ-GT和TB次之。γ-GT、TB及CB经平行试验,敏感性和阴性预测值均提高至100%;经系列试验,特异性提高至90.4%,阳性预测值提高至87.5%。结论：γ-GT、TB和CB水平对BA有一定诊断价值,平行试验阳性者更需积极做影像学检查。［中国当代儿科杂志,2009,11（12）：953-956］     

Ӥ�������ۺ���Ӧ��΢��̬���ڼ��󳦵���Ⱥ����֭�ɷֵ����˫ä�����ٴ��о�

??Abstract??Objective??To explore influence of probiotics ??lactobacillus acidophilus?? on the intestinal flora and bile composition in infantile hepatitis syndrome??IHS??. Methods??Researchers randomly assigned 60 IHS children who were the patients in Tongji Hospital from Mar.2002 to May 2008 into treatment group and control group in desired random number table. The treatment group received Lactobacillus LB sachet ??two times a day?? a packet each time??15 days as a course of treatment??and the control group received freeze dried culture medium??both on the basis of basic therapies such as UDCA??three times a day?? 25 mg each time?? and liver protection therapy. Total bilirubin??TB????direct bilirubin??DB????alanine aminotransferase??ALT???? γ-glutamyltranspeptidase??γ-GT???? totle bile acid??TBA?? of serum and intraduodenal drain??bile?? were observed before and after the treatment. SPSS 10.0 software was used to analyze the data. Results??After two treatment courses?? the effective rates were 92.86% in the treatment group and 74.07% in the control group respectively. Significant differences were observed between the two groups??P < 0.05??. Compared with the control group??the levels of TB??DB??ALT??γ-GT and TBA in serum of treatment group reduced significantly??P < 0.05????and the liver size reduced obviously??P < 0.05????while the level of TB??DB??γ-GT and TBA in bile of treatment group increased significantly ??P < 0.05?? . There was no obvious side effect in the two groups. Conclusion??There is intestinal flora disturbance in infantile hepatitis syndrome. Probiotics ??Lactobacillus acidophilus?? and UDCA can increase the excretion of bile flow?? improve the function of liver?? and stabilize the balance of intestinal flora?? which has significant curative effect on infantile hepatitis syndrome ??IHS??.     

Citrin缺陷导致的新生儿肝内胆汁淤积症30例

目的 探讨Citrin缺陷导致的新生儿肝内胆汁淤积症(NICCD)患儿的临床表现和实验室检查特点.方法 选取2008年1月-2009年12月就诊于深圳市儿童医院的30例黄疸患儿(结合胆红素及未结合胆红素均升高,以结合胆红素升高为主),经常规实验室检查,结合尿气相色谱质谱分析、基因检测诊断为NICCD.对确诊患儿的临床表现、常规实验室检查、尿异常代谢产物、基因检测等进行分析.结果 30例NICCD患儿中93%(28/30例)患儿γ-谷氨酰转肽酶升高、43%(13/30例)患儿ALT升高、80%(24/30例)患儿AST升高、57%(17/30例)患儿ALP升高,4例存在低清蛋白血症,20例部分活化的凝血酶原时间延长,70%(21/30例)的患儿并巨细胞病毒感染.23例有高氨血症,97%(29/30例)的患儿甲胎蛋白升高.尿气相色谱质谱(GC-MS)分析显示28例(93%)患儿半乳糖增高,1例丝氨酸/苏氨酸比例倒置,1例丙氨酸及苏氨酸升高,6例并4-羟基苯乳酸增高.确诊后给予去乳糖饮食,所有患儿治疗1周黄疸明显消退,血总胆红素降低50%以上.结论 以结合胆红素升高为主的黄疸患儿鉴别诊断应考虑到NICCD,确诊后应早期给予饮食干预;合并巨细胞病毒感染的病例较多见,需注意区分何种因素占主导地位,必要时联合更昔洛韦抗病毒治疗.     

婴儿胆汁淤积症142例病因及常见临床诊断方法评价

目的　分析婴儿胆汁淤积症病因构成,探讨常用临床诊断方法在诊断婴儿胆道闭锁（BA）中价值。方法　选择2012-01-01-2016-11-30福建医科大学附属第一医院儿科住院治疗的142例婴儿胆汁淤积症患儿为研究对象,回顾分析其临床表现、实验室检查、影像学检查及诊断。结果　99例（69.7%）明确诊断,其最常见病因依次为BA、巨细胞病毒感染、Citrin蛋白缺乏;37例（26.1%）诊断为特发性婴儿肝炎,6例（4.2%）病因未明;BA组排陶土样便例数高于非胆道闭锁（NBA）组,血γ-GT明显高于NBA组（P均＜0.01）。以排陶土样便为评判标准诊断BA敏感度、特异度及准确度分别为86.8%、63.4%及69.8%,γ-GT≥300 U/L为评判分界点诊断BA灵敏度、特异度及准确度分别为65.2%、91.8%、83.3%。81.2%BA患儿胆囊窝未探及胆囊声像或胆囊体积小,NBA组仅13.8%（P＜0.01）,以胆囊窝未探及胆囊声像或胆囊体积小为评判标准诊断BA敏感度、特异度及准确度分别为68.8%、90.8%及84.9%。95.0%BA患儿未见胆囊显影或肠道没有放射性浓聚,5.0%为延迟胆囊显影或肠道有放射性浓聚,NBA组50.0%未见胆囊显影或肠道没有放射性浓聚,50.0%为延迟胆囊显影或肠道有放射性浓聚,两组间比较差异有统计学意义（P＜0.01）;以放射性核素肝胆显像未见胆囊显影或肠道没有放射性浓聚为评判标准诊断BA敏感度、特异度及准确度分别为95%、50%及68.0%。结论　婴儿胆汁淤积症常见病因为BA、特发性肝炎、巨细胞病毒感染、Citrin蛋白缺乏等;如出现排陶土样便、肝肋下≥3 cm,血γ-GT≥300 U/L,腹部超声胆囊窝未探及胆囊声像或胆囊大小形态异常,核素显像未见胆囊显影或肠道没有放射性浓聚,需高度怀疑婴儿胆道闭锁。     

Establishment of age- and gender-specific pediatric reference intervals for liver function tests in healthy Han children

Background

The development and growth of children influence values of liver function tests. This study aims to establish age- and gender-specific pediatric reference intervals of liver function among Han children in Changchun, China.

Methods

A total of 1394 healthy Han children, aged 2–14 years, were recruited from communities and schools with informed parental consent in Changchun. The levels of serum alanine aminotransferase (ALT), aspartate aminotransferase (AST), γ-glutamyltransferase (GGT), alkaline phosphatase (ALP), total protein (TP), albumin (ALB), total bilirubin (TBIL) and direct bilirubin (DBIL) were measured on Hitachi 7600-210 automatic biochemical analyzer. The age- and gender-specific reference intervals were partitioned using Harris and Boyd’s test and calculated using nonparametric rank method. The pediatric reference intervals were validated in five representative hospitals located in different areas in Changchun.

Results

All the analytes required some levels of age partitioning. Proteins (TP, ALB) and bilirubins (TBIL, DBIL) required no gender partitioning. In contrast, considerable gender partitioning was required for serum ALT, AST, GGT, and ALP. TP, TBIL, and DBIL showed steady increases, and AST showed apparent decreases over time, whereas ALT, GGT, ALP, and ALB demonstrated complex trends of change. ALT and GGT increased sharply in males from 11 to 14 years old. However, ALP declined in females from 13 to 14 years. All five laboratories passed the validation of reference intervals.

Conclusions

There were apparent age or gender variations of the reference intervals for liver function. When establishing pediatric reference intervals, partitioning according to age and gender is necessary.

     

���ͳ������ض�Ժ�ڻ���԰�Ѫ֢Χ�����ط���

??Abstract??Objective??To explore the perinatal risk factors and clinical features of nosocomial neonatal sepsis in very-low-birth-weight??VLBW ??infants. Methods??Twenty-nine VLBW infants with nosocomial sepsis and 108 VLBW infants with non-sepsis born in hospital from January 2005 to June 2008 composed the study population. Their maternal?? perinatal??or postnatal variables were retrospectively analyzed. SPSS11.0 software was used to do statistical tests and multiple logistic regression. Results??Among 137 VLBW infants ??twenty-nine were nosocomial neonatal sepsis ??21.16% ??29/137??. The mortality rate was 13.79%??4/29????The incidence of nosocomial sepsis was increased with the gestational age and birth weight decreased. Gram-negative bacteria accounted for 70% of microbes?? Klebsiella pneumoniae and Escherichia coli were the most common bacterial pathogen causing nosocomial infection?? mainly extended spectrum betalactamase producing enterobacteriaceae ??ESBLs??. Birth weight≤1000g was the most impotant risk predictor of nosocomial neonatal sepsis??incidence of NBSIs was 75%??6/8????OR 7.56??4.35??14.24??. The risk factors were in the order of percutaneously inserted central catheter ??PICC???? anaemia??necrotizing enterocolitis??NEC?? and apnea . Conclusion??Nosocomial neonatal sepsis is a common problem and the main cause of late-onset death among very-low- birth-weight ??VLBW?? infants. This nosocomial infection should be lowered by active management.     

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。     

��ͯ�Ǿƾ���֬���Ը����ٴ��벡���ص����

??Abstract??Objective To explore relation skip between clinical and liver pathological characteristics of nonalcoholic steatohepatitis in children. Methods The data of 105 cases of nonalcoholic steatohepatitis children??who had been hospitalized from January 2008 to December 2012?? were summarized and analyzed. Results ??1??About 92.38% of all the group were male??11??13 years old were the most??46.67% were adiposity??0.95% had hypertension??and 33.33% had family history. ??2??The patients ALT averaged 224.90±115.55 μ/L??AST 118.57±64.38 μ/L. About 50.48% of all TG was above normal??2.86% had diabetes?? and 22.86% of insulin was above normal. About 83.81% had fatty liver by ultrasonogrophy. ??3??Liver pathology: 56.19% of all had F≥3??29.53% of all G≥2??20.95% of all S≥3. NAS score 5??6 cent accounted for 41.90% of all??7??8 cent for 58.10% of all. The group of patients with F≥3 were higher than the group of F??3 in average BMI??AST and the percentage of hyperglyceride and S≥3.??4??Liver fibrosis logistic analysis revealed that liver F grade and BMI affected liver fibrosis in NASH children. Conclusion NASH is more common in boys??and 11??13 years old is more. The group of patients F≥3 had higher than the group of F??3 in average BMI??AST and the percentage of hyperglyceride??S≥3??liver F grade and BMI affect liver fibrosis.     

ALL-2005��������Ӥ�׶������ܰ�ϸ����Ѫ�� ��Ч���о�

??Abstract??Objective To evaluate the therapeutic effectiveness and prognostic factors of ALL in infants treated with ALL-2005 protoco1. Methods Eighty-eight patients with ALL under 3 years of age were retrospectively studied ??who were newly diagnosed by morphologic and immunologic genetics and molecular biology ??MICM???? and were enrolled in the ALL-2005 treatment protocol from May 2005 to April 2009 in Shanghai Children’s Medical Center SCMC. Results Data were retrospectively collected since 2005 using the pediatric oncology networked database ??POND??. And altogether 88 children under 3 years of age with ALL received treatment in SCMC in the following four years. Among the 88 cases of ALL?? 61 ??69.32%?? were males and 27 ??30.68%?? were females. The median age at diagnosis was 2.08 years ??0??2.92 years??. Eighy-four were B lineage-ALL and 4 were T lineage-ALL. Thirty-six ??40.91%?? children were classified as low-risk?? 50 ??56.82%?? were standard-risk and 2 ??2.27%?? were high-risk. Eighty-one??92.05%?? children completely remitted in induction while 17 ??19.32%?? children relapsed. After a median follow-up of 33 months ??1.0??68.2 months???? the estimated 5-year event free survival ??EFS??rate and overall survival rate were 69.51% and 82.95%?? respectively. Conclusion The estimated 4-year EFS of ALL in infants has been close to 70%?? the therapeutic effectiveness of the ALL-2005 protocol for ALL in infants is satisfactory.     

胆道闭锁术中胆汁流量及胆汁成分的观察分析

目的 探讨胆道闭锁术中胆汁流量及成分变化与预后的关系.方法 本组对27例BA患儿行Kasai手术时,采用5 ml气体采样管收集和测量从肝门流出的胆汁.以17例胆总管囊肿患儿术中收集的胆汁作为对照组,其中测胆汁流量者7例.采用全自动生化仪检测胆汁中磷脂、总蛋白(TP)、胆汁酸(TBA)、总胆红素(TBIL)、直接胆红素(DBIL)、总胆固醇(TC)、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1～6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.结果 BA组胆汁流量显著低于对照组(P<0.05).手术日龄>60 d的BA患儿及术前血ALT>150 U/L的BA患儿术中胆汁流量均显著低于对照组(P<0.05).术中胆汁流量≥0.19 ml/min的BA患儿术后3个月退黄率显著高于术中胆汁流量<0.19 ml/min的BA患儿(P<0.05).BA组的胆汁磷脂、TBA及DBIL浓度均显著低于对照组(P<0.05);BA组的胆汁ALT浓度显著高于对照组(P<0.01).术后3个月黄疸消除者的胆汁AST及TC浓度均显著低于未消除者(P<0.05).结论 BA术中胆汁流量与预后有明显的关系,患儿术中胆汁流量越好,术后的预后越好,提示术中胆汁流量可以更早地预测患儿的远期效果.BA胆汁成分的变化说明BA患儿的肝功能较差,胆汁中磷脂、TBA和DBIL浓度较低可能是肝脏分泌功能较差的表现.胆汁中的AST和TC浓度与预后有关,胆汁AST和TC浓度较低的BA患儿术后预后较好,可能是由于肝功能较好和胆汁粘度较低利于引流所致.