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...     

胆管闭锁婴儿术前可溶性黏附分子的状况

目的:肝脏和残余胆管中的细胞间黏附分子(ICA M)的表达是胆管闭锁(BA)的炎症性特征表现。循环中这种可溶性黏附分子(SAM)的水平能够反映肝内疾病,也是评价BA的一个有用的辅助因子。研究设计:在实施肝门-肠吻合术时,对BA患儿血清ICA M-1(sICA M-1)、血清血管细胞黏附因子(sVCAM-     

白介素13在胆管闭锁和先天性胆管扩张患儿外周血及肝组织中的表达

【】 目 的 研究胆道闭锁(BA)患儿血清及肝脏中白介素-13(IL-13)的表达及意义。方 法 选取13例BA与10例胆管扩张症(CBD)患儿血清和肝脏标本进行对比研究。应用酶联免疫吸附试验检测血清中IL-13的表达水平。荧光定量RT-PCR技术和免疫印迹技术分别检测肝脏组织中IL-13 mRNA及蛋白表达水平。结 果 BA组血清中IL-13表达明显低于CBD组(154.5±18.23 pg/ml vs. 268.2±19.30 pg/ml,p<0.01)。BA组肝组织中IL-13 mRNA表达明显低于CBD组(0.0150±0.0021 vs. 0.0320±0.0050,p=0.0220)。BA组肝组织中IL-13 蛋白表达亦明显低于CBD组(0.3446±0.0150 vs. 0.5765±0.0215,p=0.0122)。     

Factors associated with inter-institutional variations in sepsis rates of very-low-birth-weight infants in 34 Malaysian neonatal intensive care units

INTRODUCTION

This study aimed to determine whether patient loads, infant status on admission and treatment interventions were significantly associated with inter-institutional variations in sepsis rates in very-low-birth-weight (VLBW) infants in the Malaysian National Neonatal Registry (MNNR).

METHODS

This was a retrospective study of 3,880 VLBW (≤ 1,500 g) infants admitted to 34 neonatal intensive care units (NICUs) in the MNNR. Sepsis was diagnosed in symptomatic infants with positive blood culture.

RESULTS

Sepsis developed in 623 (16.1%) infants; 61 (9.8%) had early-onset sepsis (EOS) and 562 (90.2%) had late-onset sepsis (LOS). The median EOS rate of all NICUs was 1.0% (interquartile range [IQR] 0%, 2.0%). Compared with NICUs reporting no EOS (n = 14), NICUs reporting EOS (n = 20) had significantly higher patient loads (total live births, admissions, VLBW infants, outborns); more mothers with a history of abortions, and antenatal steroids and intrapartum antibiotic use; more infants requiring resuscitation procedures at birth; higher rates of surfactant therapy, pneumonia and insertion of central venous catheters. The median LOS rate of all NICUs was 14.5% (IQR 7.8%, 19.2%). Compared with NICUs with LOS rates below the first quartile (n = 8), those above the third quartile (n = 8) used less intrapartum antibiotics, and had significantly bigger and more mature infants, more outborns, as well as a higher number of sick infants requiring ventilator support and total parenteral nutrition.

CONCLUSION

Patient loads, resuscitation at birth, status of infants on admission and treatment interventions were significantly associated with inter-institutional variations in sepsis.     

超声检测胆囊和十二指肠液引流检查对婴儿黄疸的鉴别诊断意义

目的　探讨超声胆囊和十二指肠液引流检查对婴儿持续性黄疸的鉴别诊断意义。方法　应用实时超声仪观察哺乳前后婴儿胆囊大小变化 ,应用婴儿十二指肠引流术收集胆汁。结果　经随访黄疸消退的 30例婴儿肝炎综合征哺乳前胆囊最大断面积为 (2 .0 7± 0 .96 )cm2 ,哺乳后 30min为 (0 .92± 0 .12 )cm2 ,胆囊收缩率为 (5 4 .5± 16 .6 ) % ;十二指肠液胆红素≥12 .5 μmol/L ,胆汁酸阳性。经手术证实的 32例胆道闭锁哺乳前胆囊最大断面积为 (0 .81± 0 .14 )cm2 ,哺乳后 30min为(0 .6 7± 0 .11)cm2 ,胆囊收缩率为 (17.1± 11.5 ) % ,十二指肠液无胆汁。两组胆囊收缩率对比具有极显著性差异 (P <0 .0 1)。结论　超声检测哺乳前后胆囊大小变化及胆囊收缩率结合十二指肠液检查 ,能准确鉴别婴儿肝炎综合征与胆道闭锁。     

婴儿肝外胆道闭锁肝组织MRP2及FXR蛋白的表达及其意义

目的 探讨肝外胆道闭锁(EHBA)婴儿肝组织中多药耐药相关蛋白2(MRP2)、法尼醇受体(FXR)蛋白的表达及其与胆汁淤积的关系.方法 将临床和病理诊断为EHBA的20例患儿肝组织作为EHBA组,肝移植手术中成人供体的肝组织10例作为对照组,比较两组患者肝功能指标;采用免疫组织化学法检测肝组织中MRP2、FXR的表达情况,并进行病理图像分析.结果 EHBA组患儿血清总胆红素、直接胆红素、丙氨酸转氨酶、天冬氨酸转氨酶及γ-谷氨酰转肽酶(γ-GT)浓度明显高于对照组(P<0.05).对照组和EHBA组肝组织MRP2 蛋白吸光度(A)值分别为0.16±0.04和0.10±0.02,对照组和EHBA组     

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。     

胆道闭锁99例临床诊疗分析

目的:分析胆道闭锁诊疗及预后情况,探讨胆道闭锁Kasai手术的意义。方法:回顾性分析郑州大学第一附属医院小儿外科2008年8月至2013年4月收治的99例胆道闭锁患儿的病例资料,定期门诊复查并电话随访,分析所有患儿的手术及存活情况。结果:20例未行Kasai手术患儿中,3例存活,均有肝硬化、腹水等症状;77例行Kasai手术的患儿中,46例存活,两组差异有统计学意义(P<0.05)。2例行胆囊十二指肠吻合术,均存活。行Kasai手术的存活患儿中,手术日龄在90 d以内(包含90 d)的患儿30例存活,手术日龄>90 d患儿16例存活,差异有统计学意义(P<0.05)。结论:根据临床表现、影像学检查、腹腔镜探查并活检等有助于胆道闭锁的诊断。早诊断、早治疗,及时行Kasai术可以延长胆道闭锁患儿的生命,为肝移植赢得时间。     

Forty-four living donor liver transplantations for children with biliary atresia

<正>Objective To observe the outcomes of living donor liver transplantation ( LDLT) for children with biliary atresia ( BA) and to summarize clinical experiences. Methods Forty - four BA patients ( 26 boys and 18 girls) underwent LDLT between October 2006 and December 2010. Mean ( SD) and median ( range) age at     

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.     

MR Cholangiography and Dynamic Examination of Duodenal Fluid in the Differential Diagnosis between Extrahepatic Biliary Atresia and Infantile Hepatitis Syndrome

The common causes of infantile persistent choles-tatic jaundice are infantile hepatitis syndrome (IHS) and extrahepatic biliary atresia (EHBA)[1]. The differential diagnosis between IHS and EHBA is very difficult be-cause there is considerable clinical, biochemical, and histopathologic overlaps between them. Magnetic reso-nance cholangiography (MRC) is a newly-established non-invasive modality used for the diagnosis of EHBA in infants. Their results varied on the accuracy, sensitiv-ity…     

同卵双胞胎胆道闭锁患儿染色体核型和全外显子组测序分析

目的:以临床表型不同的同卵双胞胎胆道闭锁（BA）患儿为研究对象,分别进行染色体G显带核型分析和全外显子组测序,检测该组双胎儿是否存在染色体核型异常和差异基因型,初步探讨胆道闭锁发生的遗传学背景。方法:采集3对同卵双胞胎BA患儿外周静脉血作为实验样本,分别进行染色体G显带核型分析和全外显子组测序分析。结果:临床表型不同的同卵双胞胎BA患儿,其染色体G带核型分析结果均无异常,为46,XX或46,XY。全外显子组测序结果显示,未检测到该组同卵双胞胎BA患儿的任何差异基因型。结论:同卵双胞胎BA患儿具有不同的临床表型,其染色体G显带核型正常,提示胆道闭锁的发生与染色体异常无关;双胎儿全外显子组测序检测不存在差异基因型,提示胆道闭锁不是单纯的遗传性疾病,可能与遗传表型或外显率以及其他因素等有关。     

Pattern of neonatal septicemia in a Malaysian maternity hospital

Over a 12 months period, out of 25,411 livebirths, 155 neonates (6.1 per 1000 livebirths) had proven septicemia by blood culture. The mortality rate was 26.5%. Septicemia was more common among the very low birthweight and preterm neonates of gestation of 30 weeks or less. 45.8% of the septicemia occurred during the first 48 hours of life. Staphylococcus epidermidis was the most common causative organism. However, mortality was highest among neonates who acquired multiresistant nosocomial infection during the later part of neonatal life.     

Malaysian mothers' knowledge & practices on care of neonatal jaundice

This study aimed to determine the gaps of knowledge and practices of care of neonatal jaundice among Malaysian mothers. It was a cross sectional study of 400 mothers who attended the obstetric clinics or were admitted to the obstetric wards of a general hospital. They were surveyed with a structured set of questionnaire. The results showed that a majority (93.8%) of them knew about neonatal jaundice, and 71.7% knew that jaundice lasting more than 2 weeks was abnormal. However, only 34.3% of them were aware that jaundice appearing during the first 36 hours of life was abnormal. Less than 20% knew about glucose-6-phosphate dehydrogenase deficiency and that fetal-maternal blood group differences could cause jaundice. Although 71.7% and 69.7%, respectively, of the mothers knew that severe jaundice could cause death and brain damage, only 38.4% of them were aware that severe jaundice could result in hearing impairment. A very low proportion (27.1%) of them was aware that putting jaundiced infants under the direct sun could result in dehydration and worsening of jaundice. Out of a maximum score of 15, the mean maternal knowledge score was 7.4 (95% confidence intervals: 7.1, 7.7). Majority (83.1%) of the multiparous mothers with a past history of having children developing neonatal jaundice (n = 154) practiced placing their infants under the direct sun. This study revealed that there was a wide knowledge gap among Malaysian mothers on care of neonatal jaundice. Placing infants under the direct sun was still a common practice.